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REVISION NOTES IN NEUROSURGERY FOR MBCHB STUDENTS

NEUROSURGERY CLERKSHIP KENYATTA NATIONAL HOSPITAL


Course Description:
Students are required to take neurological surgery clerkship during their third and fifth
year. The neurological surgery clerkship emphasizes the development of skills in
neurological examination, functional neuroanatomy, practical interpretation of
neuroimaging and the identification of emergent neurological conditions, as well as the
medical and surgical management of cranial, spinal and peripheral nerve disease.
Course Objectives: At the end of this rotation, students will be able to
acquire skills in neurological examination, functional neuroanatomy, practical
interpretation of neuroimaging and the identification of emergent neurological conditions,
as well as the medical and surgical management of cranial, spinal and peripheral nerve
disease.
take a patient history including pertinent neurological review of systems, past medical
history, family history and social history .
reliably perform a general neurological examination including mental status, cranial
nerve, cerebellar, Motor, Sensory, and reflex subcomponents
calculate the Glasgow Coma Score (GCS) for any given patient
calculate the functional status of any given patient according to the Karnofsky
Performance Scale
identify and neuroanatomically localize common neurological deficits including lobar
lesions, brain stem lesions, myelopathy, radiculopathy, and peripheral nerve deficits
become familiar with the common neurological diseases that must be considered in the
differential diagnosis of patients presenting with varying combinations of, and time
courses for, neurological symptoms and examination findings
become familiar with the various tests that are used for neurological evaluation, when
these tests are appropriate, as well as their limitations
identify the presence or absence of skull fracture, intracranial hemorrhage,
hydrocephalus, and/or a lesion causing mass effect on a cerebral neuroimage
identify the presence or absence of spinal fracture, spinal cord compression, or a
significantly herniated disc on a spinal neuroimage .
become familiar with the neurocritical care concepts and monitoring techniques for
measuring intracranial pressure, cerebral perfusion pressure, and cerebral artery
vasospasm, syndrome of inappropriate antidiuretic hormone release, cerebral salt wasting
syndrome, diabetes insipidus, and vasogenic cerebral edema.
become familiar with the medical and surgical management of neurological emergencies,
including acute spinal cord compression, elevated intracranial pressure, intracerebral
hemorrhage, seizures and stroke.
become familiar with the basic types of operations performed to assist in the diagnosis
and treatment of patients with neurological disease.
become familiar with ethical and quality of life issues including, loss functional
independence, alterations in body image, issues surrounding limitation or withdrawal of
care decisions, informed consent, and organ donation, that are inherent in major
neurological illness and injury as well as neurological surgery
Key Topics:

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Neuroanatomical localization
Alteration in consciousness
Functional and quality of life impact of disease and injury
Ethical implications of surgery, as well as neurological disease and injury
Basic practical interpretation of neuroimaging
Recognition and management of neurological emergencies
Traumatic brain and spine/spinal cord injury along with neurocritical care
Cerebrovascular disease
Neuro-Oncology
Degenerative spine disease
Competencies:
Neurological history
Neurological examination
Neuroanatomical localization
Common tests utilized to evaluate neurological disease
Basic interpretation of neuroimaging
Differential diagnosis of neurological presentations and findings
Identification and treatment of neurological emergencies
Role of surgery in neurological disease
Appreciation & familiarity with concepts & techniques unique to neurocritical care
Professional and ethical approach to patients and families
Attitudes and Commitments:
An understanding of the integrity, commitment, and work ethic required to become an
effective and successful neurological clinician.
An organized, rational, systematic and thorough approach to patient evaluation and
diagnosis.
The need and means to effectively manage the complexities of our health care system,
policies and procedures in order to maximize the potential outcome for patients in the
setting of urgent medical conditions.
An understanding of the importance of multidisciplinary and multi-departmental
integration and cooperation for optimizing care for patients with complex disease.
An understanding of the importance of new and often expensive technology in advancing
the diagnosis and treatment of neurological disease.
An understanding of the critical importance of compassion, effective communication, and
the highest ethical standards in assisting patients and families with the major issues and
decisions surrounding high-risk surgery as well as major neurological disease and injury.
An understanding of the great promise that advancing basic neuroscience holds for
further advancements in translational clinical neuroscience.
A willingness to emulate the neurosurgical faculty when it comes to integrity, work ethic,
professional and ethical behavior, and personal commitment to learning as well as
individual patient care.
Educational Activities:
The neurological surgery clerkship is fourteen weeks in length (3rd and 5th year). It
includes daily inpatient ward and neurosurgical intensive care unit service exposure for
work and educational rounds and patient care. On the inpatient service, each student will
be fully integrated into the overall neurosurgical team. They will participate in inpatient

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and emergency room consultations during regular working hours, and will follow and
write progress notes on cases assigned to them. Each group will be assigned to one
neurosurgical faculty mentor. They will meet with this mentor as a group at least once per
week. They will accompany that mentor to his or her outpatient ambulatory clinic as well
as the operating room for any scheduled surgical case. On days where their mentor is not
scheduled for clinic or surgery, the students may attend the clinic or surgery of one of the
other department consultants after having completed their inpatient service
responsibilities. Thursday is an academic day for the department and attendance at all
teaching conferences for the day is mandatory, as is attendance at the weekly meeting
with the rotation mentor. The assigned faculty mentor will be responsible for completing
the student evaluations for each rotation with input from the postgraduate residents and
the other neurosurgical faculty. A post-rotation test will be administered to assess the
effectiveness of the rotation in meeting our educational goals and our responsibility to
our students.
Students should review the elements of the neurological history and examination and
basic neurology and neurosurgery from the lecture notes (Prof Mwang’ombe).
Clinical Responsibilities of the Student:
Procedures to be Learned by the Student: lumbar puncture, arterial lines, intravenous
lines (central and peripheral), intracranial monitors and ventriculostomies, as well as
basic surgical technique
Official Evaluation Policy:
The student will be evaluated on attendance, participation, knowledge base, clinical
skills, motivation, professionalism, and interpersonal skills.

Course Hours Summary:


20 Patient care actives
10 Laboratory & Imaging
6 Lecture
12 Conference
24 Clinic
8 Grand Rounds
28 Inpatient
46 Preceptorship (surgery)
6 Small Group
28 Ward Rounds
2 Exams
162 Total (including night calls)

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Neurosurgical Division Kenyatta National
Hospital Clinical Teaching Program

Monday: Monday:
8:00 am – 11:00 am 2:00pm- 5:00 pm
WR/PG Teaching; PW, PM,DO,MQ NSOPC (No. 24)
MBChB 5-Clinical Teaching
NM, JK, PA, CM, PM
Tuesday: Tuesday:
8:00 am-11:00 am 2:00 pm-5:00 pm
MBChB 5-Wd Teaching: (JK). NSOPC (No. 24)
WR/PG teaching; JK DO, PW, PL, MQ.
Theatre whole day: NM, PA, PM Theatre whole day: NM,PA, PM
Wednesday: Wednesday:
8.00 am-9.00 am: Theatre-whole day: DO, PW,MQ
PGyr 2 PoS, (NM).
9:00 am – 10:00 am:
MBChB 3-lecture: (NM)
10:00 am-11:00 am:
MBChB 3-wd teaching (PA/DO)
WR/PG teaching; PA/DO
Theatre-whole day: DO,PW, MQ
Thursday: Academic Day Thursday: Academic Day
8:00 am-9:00 am 2:00 pm-3:00 pm
Neuroradiology Conference (Rad. Dept.) Case Conference
9:00 am-11:00 am Grand Round (NM)
MBChB 5-wd teaching (CM)
11 am-1 pm: Neurosurgery lectures,
Journal Club/ M&M conference (Surg
Dept)
Friday: Friday:
7.00 am-8.00 am
Neuropathology Conference
(Dept. Path) Theatre-whole day (PL, CM, JK)
Theatre-whole day ( PL, CM, JK)
WR; ward round. PG; post graduate. M&M; mortality and morbidity. wd; ward. PoS;
principles of surgery. PGyr; post grad year.
NM; Prof Mwang’ombe. PA; Dr. Akuku. CM; Dr. Musau. DO; Mr. Olunya. JK; Dr.
Kiboi. PL; Dr. Lubanga. PW; Dr. wanyoike. PM; Dr. Mwangi. MQ; Mr. Qureshi.

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Neurological History and Physical Examination
"From the brain and the brain only arise our pleasures, joys, laughter and jests, as well as
our sorrows, pains, griefs, and tears.... These things we suffer all come from the brain,
when it is not healthy, but becomes abnormally hot, cold, moist or dry."
—Hippocrates
Taking the patient's history is traditionally the first step in virtually every clinical
encounter. A thorough neurologic history allows the clinician to define the patient's
problem and, along with the result of physical examination, assists in formulating an
etiologic and/or pathologic diagnosis in most cases.
Solid knowledge of the basic principles of the various disease processes is essential for
obtaining a good history. As Goethe stated, "The eyes see what the mind knows." To this
end, the reader is referred to the literature about the natural history of diseases. The
purpose of this article is to highlight the process of the examination rather than to provide
details about the clinical and pathologic features of specific diseases.
The history of the presenting illness or chief complaint should include the following
information:
Symptom onset (eg, acute, subacute, chronic, insidious)
Duration
Course of the condition (eg, static, progressive, or relapsing and remitting)
Associated symptoms, such as pain, headache, nausea, vomiting weakness, and seizures
Pain should be further defined in terms of the following:
Location
Radiation
Quality
Severity or quantity
Precipitating factors
Relieving factors
Important miscellaneous factors of the history include the following:
Results of previous attempts to diagnose the condition
Any previous therapeutic intervention and the response to those treatments
A complete history often defines the clinical problem and allows the examiner to proceed
with a complete but focused neurologic examination.
Neurologic Examination
The neurologic examination is one of the most unique exercises in all of clinical
medicine. Whereas the history is the most important element in defining the clinical
problem, neurologic examination is performed to localize a lesion in the central nervous
system (CNS) or peripheral nervous system (PNS). The statement has been made,
"History tells you what it is, and the examination tells you where it is." The history and
examination allow the neurologist to arrive at the etiology and pathology of the condition,
which are essential for treatment planning.
Unlike many other fields of medicine in which diseases are visible (eg, dermatology,
ophthalmology) or palpable (eg, surgery), neurology is characterized by conditions that
may be detected only by applying specific examination techniques and logical deduction,
except when telltale cutaneous markers or other stigmata (see Media files 1-8) suggest

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the diagnosis. Considerable insight and intuition are required to interpret the symptoms
and signs observed during neurologic examination. These features make the neurologic
history and physical examination both challenging and rewarding.
A properly performed neurologic examination may take 90 minutes or even longer for the
novice. Experienced neurologists take substantially less time and can frequently grasp the
essential features of a clinical condition quickly. What might appear to be a complex
problem of localization for the referring physician may turn out to have a simple
explanation, and the neurologic consultation may help to avoid extensive testing.
Neurologic examination in the year of imaging
With the advent of CT scanning in the early 1970s, the future clinical role of the
neurologist was questioned. During one of his visits to the United States, Dr. McDonald
Critchley was asked what he thought would be the future of neurology in the era of CT.
His answer was most enlightening: "CT scanning will take away the shadows of
neurology, but the music will still remain." These prophetic words still ring true despite
the advent of MRI, positron emission tomography (PET), and functional neuroimaging of
all types.
It has been said that "neurology owes more to its disorders than those disorders owe to
neurology." This is because much knowledge has come from previous observations of
neurologic conditions, because the eponyms for the diagnoses were sometimes long, and
because so little was previously offered in the terms of cures such that the specialty was
ridiculed as one that was "long on diagnosis and short on treatment." Fortunately,
technologic advances have changed that perception.
Steps in the neurologic examination
In examining a patient, abnormalities of function lead to localization and, eventually, to
the pathophysiology. For the purpose of simplicity, the neurologic examination is divided
into several steps. When mastered, these steps become second nature to the examiner, and
the process of evaluating the patient proceeds smoothly, even though the steps are not
always necessarily performed in the same order. These steps include the following:
Higher functions
Cranial nerves (CNs)
Sensory system
Motor system
Reflexes
Cerebellum
Meninges
System survey
Tools required
In addition to the stethoscope and the usual office supplies (eg, gloves, tongue
depressors), the neurologist should have an ophthalmoscope, a reflex hammer, and a
tuning fork.
A pin (Wartenberg) wheel was once a favorite tool of many neurologists because it was
easy to use for sensory (pinprick) testing. Unless it is disposable (commercially
available), this wheel is no longer recommended because of the risk of transmitting
infection. The use of sterile safety pins (to be discarded after each use) is recommended.
The final section of this article includes a Definition of Terms.
Examination of the Higher Functions

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Higher functions include gait, speech, and mental status. These are referred to as higher
functions because human bipedal gait, receptive and expressive speech, and cognitive
function are more sophisticated than similar functions of any other member of the animal
kingdom.
Gait
Gait is the attitude of a person in the upright position. Abnormal types are described
below.
Hemiparetic gait
In hemiparesis, facial paresis may not be obvious. In mild cases, subtle features of facial
paralysis (eg, flattening of the nasolabial fold on 1 side compared to the other, mild
asymmetry of the palpebral fissures or of the face as the patient smiles) may be sought.
The shoulder is adducted; the elbow is flexed; the forearm is pronated, and the wrist and
fingers are flexed. In the lower extremities, the only indication of paresis may be that the
ball of the patient's shoe may be worn more on the affected side.
In severe cases, the hand may be clenched; the knee is held in extension and the ankle is
plantar flexed, making the paralyzed leg functionally longer than the other. The patient
therefore has to circumduct the affected leg to ambulate.
In hemiplegic patients in whom all the paralysis is on the same side of the body, the
lesion is of the contralateral upper motor neuron. In most cases, the lesion lies in the
cortical, subcortical, or capsular region (therefore above the brainstem). In the alternating
or crossed hemiplegias, CN paralysis is ipsilateral to the lesion, and body paralysis is
contralateral. In such cases, CN paralysis is of the lower motor neuron type, and the
location of the affected CN helps determine the level of the lesion in the brainstem.
Therefore, paralysis of CN III on the right side and body paralysis on the left (Weber
syndrome) indicates a midbrain lesion, whereas a lesion of CN VII with crossed
hemiplegia (Millard-Gubler syndrome) indicates a pontine lesion, and CN XII paralysis
with crossed hemiplegia (Jackson syndrome) indicates a lower medullary lesion.
Ataxic gait
In ataxia, the patient spreads his or her legs apart to widen the base of support to
compensate for the imbalance while standing or walking. In severe cases, patients stagger
as they walk. The heel-to-toe or tandem walking maneuvers and standing on 1 leg
uncover subtle forms of ataxia.
Ataxia results from midline lesions of the cerebellum and may be isolated or associated
with other cerebellar findings (see Cerebellar signs). When the lesion is unilateral, the
patient may veer to the side of the lesion. With bilateral cerebellar involvement, the
patient may fall to either side.
Shuffling gait
The individual takes short steps to the point of practically not moving forward or making
little progress. In other words, the patient appears to shuffle his or her legs rather than put
them forward. In some patients, the steps (albeit short) and pace may vary with a
tendency for the patient to accelerate (festinating gait) as he or she walks. Both types are
seen in Parkinson disease and may be associated with other extrapyramidal signs.
Steppage gait
In steppage (high-stepping, slapping), the individual takes high steps as if climbing a
flight of stairs while walking on a level surface. This peculiar gait pattern results from the
patient trying to avoid injury to the feet (from dragging them) by stepping high. However,

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as the patient puts the feet down 1 by 1, they slap the ground, hence the description of a
foot-slapping gait. This is 1 condition that can be diagnosed even before the patient enters
the room because the sound is so characteristic.
Steppage gait is seen in chronic peripheral neuropathies and can be the result of the
functional elongation of the legs due to bilateral drop foot.
Spastic or scissor gait
In this condition, the legs are held in adduction at the hip and the thighs rub against each
other as the patient walks. Spasm of the inner thigh muscles also occurs. If the spasm is
severe, with each advancing step the knees tend to slide over each other like the blades of
a pair of scissors. This is typically seen in cerebral diplegia, a form of cerebral palsy.
Antalgic gait
Patient favors the affected painful (usually lower) extremity and walks, putting weight on
the normal leg. The hand held over hip on the affected side is typical in patients with
radicular pain.
Speech
Speech enables communication between individuals. Abnormalities include dysphonia,
dysarthria, and dysphasia or aphasia.
Dysphonia or aphonia
Dysphonia is the impairment or inability to phonate. As a result, the voice becomes
hoarse. In extreme cases, it is absent, and the patient is mute.
The most frequent cause of this problem is the common cold, which results in dysphonia
due to inflammation of the larynx. Dysphonia may also occur in patients with
hypothyroidism, as a result of thickening of the vocal cords from amyloid deposits.
Neurologic causes include unilateral recurrent laryngeal nerve paralysis and lesions of the
vagus nerve. Intermittent hoarseness may affect patients with vagus nerve stimulator
implants, which are used for the treatment of certain medically intractable forms of
epilepsy (MIE) and pharmacoresistent depression (PRD).
Dysarthria or anarthria
Dysarthria is the inability to articulate spoken words. The quality of oration is impaired,
but the content remains intact (eg, slurred speech). The patient's ability to understand and
synthesize speech remains intact. It results from paralysis of pharyngeal, palatal, lingual,
or facial musculature. It also is observed with cerebellar lesions and/or disease (eg,
scanning or staccato speech).
Dysphasia or aphasia
In dysphasia, the ability to process language is impaired, resulting in an inability to
understand (ie, receptive or sensory or Wernicke aphasia), transfer signals from the
Wernicke to the Broca area (ie, conduction aphasia), or properly execute speech (ie,
expressive, motor, or Broca aphasia). The combination of Broca and Wernicke aphasias
is referred to as global aphasia.

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Table 1 summarizes the essential features of common dysphasias (aphasias).
Table 1. Essential Features of Common Dysphasias
Type of Dysplasia Fluency Comprehension Naming Localization
Broca Nonfluent Intact Impaired Broca area
Wernicke Fluent Impaired Impaired Wernicke area
Conduction Fluent Intact Impaired Arcuate fasciculus
Global Nonfluent Impaired Impaired Broca and Wernicke areas
Transcortical aphasias
Another function that is impaired in all 4 of the aphasias mentioned above is repetition.
This finding is important in the diagnosis of transcortical aphasias. When repetition is
preserved in a patient with Broca aphasia, it signifies transcortical motor aphasia, and the
lesion is anterior to the Broca area. When repetition is preserved in Wernicke aphasia, it
is called transcortical sensory aphasia, and the lesion is posterior to the Wernicke area.
Transcortical mixed aphasia and global aphasia are similar except for the preservation of
repetition, and results from combined lesions anterior to the Broca and Wernicke areas,
respectively.
Mental status
Mental status evaluation includes testing of memory, orientation, intelligence, and the
other aspects of the patient's psychic state. Only the first 3 are discussed here. When overt
symptoms or signs of a psychic disturbance are present, psychiatric evaluation should be
considered.
Memory
Memory is the ability to register and recall prior sensory input. Recent and remote
memory functions are differently affected depending on the disease process. Remote
memory is relatively preserved in chronic dementing processes, with major disturbances
in the attention span and recent memory. On the contrary, all aspects of memory are
impaired in acute encephalopathies.
Orientation
Orientation is an individual's cognitive sense of his status in time, place, and person.
These functions are affected in the same order as they are in organic disease. In other
words, the sense of time is first to be impaired in organic dysfunction, and the sense of
person is the last to be lost. However, the order may be disturbed in psychological
dysfunction.
A patient who does not know who he or she is, but at the same time can tell the time and
is oriented in place, is more likely to have a psychological disturbance than to have an
organic etiology for the condition. Nonetheless, rare cases of isolated amnesia have been
reported.
Intelligence
Intelligence is the ability to quickly and successfully apply previous knowledge to a new
situation and to use reason in solving problems. Vocabulary, fund of knowledge,
calculations (eg, serial-7 calculations), abstraction (eg, use of proverbs), and judgment
(eg, what to do with a found wallet) are good indicators of intelligence.
Psychological disturbances

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A brief survey of the other aspects of psychological function may be helpful in revealing
abnormalities of thought process (eg, circumstantiality and tangentiality); of perception
(eg, illusions and hallucination); or of thought content (eg, delusions of grandeur).
Patients with these findings should be referred for appropriate evaluation.
Examination of the Cranial Nerves
Of the 12 CNs, some are named according to their function. Examples of these are the
olfactory (smell), optic (vision), oculomotor (eye movements), abducens (abduction of
the eye), facial (facial expression), and vestibulocochlear or statoacoustic (hearing and
balance) nerves. Others are named for their relationship to neighboring structures
(trochlear nerve), appearance (trigeminal nerve), extent of distribution (vagus nerve),
composition (spinal accessory nerve), or location (hypoglossal nerve).
Trochlear: Its midsection extends over a trochlea or pulley to reach its insertion on the
inferior aspect of the globe.
Trigeminal: The nerve divides into 3 divisions distal to the Gasserian ganglion.
Vagus: The vagabond or wanderer, it travels long distances in the body.
Spinal accessory: This nerve is composed of rootlets from the spinal cord in addition to
its medullary component.
Hypoglossal: Its course is sublingual in the neck.
Knowing the names of the CNs makes it easy to remember their function, thereby making
their examination self-evident. The following mnemonic is helpful in recalling the names
of the CNs: Oh, oh, oh; to trek and feel a great valley; ah! ha! Another is this: On old
Olympus towering tops, a Finn and German viewed some hops.
Olfactory nerve - CN I
The olfactory nerves consist of small unmyelinated axons that originate in the olfactory
epithelium in the roof of the nasal cavity; they pierce the cribriform plate of the ethmoid
and terminate in the olfactory bulb. Lesions of the nerve result in parosmia (altered sense
of smell) or anosmia (loss of smell).
The common cold is the most frequent cause of dysfunction. Dysfunction can be
associated with fractures of the cribriform plate of the ethmoid bone. Frontal lobe tumors
may compress the olfactory bulb and/or tracts and cause anosmia, but this is rare
occurrence.
Olfactory function is tested easily by having the patient smell common objects such as
coffee or perfume. Commercially available scented scratch papers may also be used.
Optic nerve - CN II
The optic nerve is a collection of axons that relay information from the rods and cones of
the retina. The temporal derivations reach the ipsilateral and the nasal derivations the
contralateral superior colliculi and the lateral geniculate bodies. From there, axons extend
to the calcarine cortex by means of the optic radiation, traversing the temporal (Myer
loop) and parietal lobes. Fibers responsible for the pupillary light reflex bypass the
geniculate body and reach the pretectal area, from where they innervate the
parasympathetic (midline) portion of the third-nerve nucleus, enabling the consensual
pupillary reflex.
The following testing is appropriate:
Acuity, by using the Snellen chart (near and distant vision)
Visual fields, by means of confrontation or perimetry if indicated

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Color, with use of an Ishihara chart or by using common objects, such as a multicolored
tie or color accent markers
Funduscopy
Lesions of the visual pathways result in blindness and pupillary abnormalities, such as the
Marcus-Gunn pupil (retinal or optic nerve disease), scotomata, quadrant or hemianopsias
(optic tract and radiation), and hemianopsias with macular sparing (calcarine cortex).
Oculomotor nerve - CN III
The oculomotor nucleus of the nerve is located in the midbrain and innervates the
pupillary constrictors; the levator palpebrae superioris; the superior, inferior, and medial
recti; and the inferior oblique muscles. Lesions of CN III result in paralysis of the
ipsilateral upper eyelid and pupil, leaving the patient unable to adduct and look up or
down. The eye is frequently turned out (exotropia). In subtle cases, patients complain of
only diplopia or blurred vision. Lesions at the nucleus of the third nerve cause bilateral
ptosis, in addition to the findings mentioned above. The exotropia seen in CN III
paralysis can be distinguished from that in internuclear ophthalmoplegia because in the
latter convergence is preserved.
Paralysis of CN III is the only ocular motor nerve lesion that results in diplopia in more
than 1 direction, distinguishing itself from CN IV paralysis (which also can result in
exotropia). Pupillary involvement is an additional clue to involvement of CN III. Pupil-
sparing CN III paralysis occurs in diabetes mellitus, vasculitides of various etiologies,
and certain brainstem lesions such as due to multiple sclerosis.
Trochlear nerve - CN IV
The nucleus of the nerve is located in the midbrain. It innervates the superior oblique
muscle, which incycloducts and infraducts the eye. Trochlear nerve typically allows a
person to view the tip of his or her nose.
An isolated right superior oblique paralysis results in exotropia to the right (R), double
vision that increases on looking to the (L), and head tilt to the right (R). The mnemonic is
R, L, R (ie, the marching rule). The rule is L, R, L for left superior oblique paralysis. This
rule and the lack of ptosis and/or pupillary involvement allow easy distinction of the
exotropia of CN IV paralysis from that seen in CN III paralysis.
Trigeminal nerve - CN V
The nucleus of the nerve stretches from the midbrain (ie, mesencephalic nerve) through
the pons (ie, main sensory nucleus and motor nucleus) to the cervical region (ie, spinal
tract of the trigeminal nerve). It provides sensory innervation for the face and supplies the
muscles of mastication.
Paralysis of the first division (ophthalmic; V1) is usually seen in the superior orbital
fissure syndrome and results in sensory loss over the forehead along with paralysis of CN
III and CN IV. Paralysis of the second division (maxillary; V2) results in loss of
sensation over the cheek and is due to lesions of the cavernous sinus; it also results in
additional paralysis of V1, CN III and CN IV. Isolated V2 lesions result from fractures of
the maxilla. Complete paralysis of CN V results in sensory loss over the ipsilateral face
and weakness of the muscles of mastication. Attempted opening of the mouth results in
deviation of the jaw to the paralyzed side.
Abducens nerve - CN VI
The nucleus of the nerve is located in the paramedian pontine region in the floor of the
fourth ventricle. It innervates the lateral rectus, which abducts the eye. Isolated paralysis

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results in esotropia and inability to abduct the eye to the side of the lesion. Patients
complain of double vision on horizontal gaze only. This finding is referred to as
horizontal homonymous diplopia, which is the sine qua non of isolated CN VI paralysis.
Paralysis of CN VI may result from increased intra cranial pressure without any lesion in
the neuraxis, and it may result in false localization if one is not aware of it.
Facial nerve - CN VII
The nucleus of the nerve lies ventral, lateral, and caudal to the CN VI nucleus; its fibers
elevate the floor of the fourth ventricle (facial colliculus) as they wind around the CN VI
nucleus. The nerve leaves the cranial cavity through the stylomastoid foramen and
innervates the muscles of facial expression and the stapedius.
Although it is considered a pure motor nerve, it also innervates a small strip of skin of the
posteromedial aspect of the pinna and around the external auditory canal. The nervus
intermedius of Wrisberg conducts taste sensation from the anterior two thirds of the
tongue and supplies autonomic fibers to the submaxillary and sphenopalatine ganglia,
which innervate the salivary and lacrimal glands.
A lower-motor-neuron lesion of the nerve, also known as peripheral facial paralysis,
results in complete ipsilateral facial paralysis; the face draws to the opposite side as the
patient smiles. Eye closure is impaired, and the ipsilateral palpebral fissure is wider. In an
upper motor neuron lesion, also known as central facial paralysis, only the lower half of
the face is paralyzed. Eye closure is usually preserved. In peripheral facial paralysis,
different types of clinical presentations are seen with nerve lesions at 4 levels, as
described below.
Lesions of the meatal or canalicular segment: Facial paralysis with hearing loss (without
hyperacusis) and loss of taste in the anterior two thirds of the tongue imply lesions in the
internal auditory canal from fracture of the temporal bone or at the cerebellopontine angle
from compression by a tumor.
Lesions of the labyrinthine or fallopian segment
Lesions that spare hearing (with hyperacusis) indicate lesions further down the course of
the nerve.
Loss of taste in the anterior two thirds of the tongue and loss of tearing imply lesions that
involve the chorda tympani and the secretomotor fibers to the sphenopalatine ganglion in
the labyrinthine segment, proximal to the greater superficial petrosal nerve.
With lesions distal to the greater superficial petrosal nerve, lacrimation is normal but
hyperacusis is still present. Geniculate lesions in this segment cause pain in the face.
Lesions of the horizontal or tympanic segment: The lesion is proximal to the departure of
the nerve to the stapedius and results in hyperacusis, loss of taste in the anterior two
thirds of the tongue, and facial motor weakness.
Lesions of the mastoid or vertical segment: Hyperacusis is present if the lesion is
proximal to the nerve to the stapedius. It is absent if the lesion is distal to the nerve to the
stapedius, and only loss of taste and facial paralysis occur. If the lesion is beyond the
chorda tympani in the vertical segment (as in lesions of the stylomastoid foramen), taste
is spared and only facial motor paralysis is seen.
Vestibulocochlear nerve - CN VIII
The vestibulocochlear or statoacoustic nerve enters the brainstem at the pontomedullary
junction and contains the incoming fibers from the cochlea and the vestibular apparatus,
forming the eighth CN. It serves hearing and vestibular functions, each of which is

12
described separately. Hearing loss may be conductive or sensorineural. Three tests help in
evaluating the auditory component of the nerve.
The Weber test involves holding a vibrating tuning fork against the forehead in the
midline. The vibrations are normally perceived equally in both ears because bone
conduction is equal. In conductive hearing loss, the sound is louder in the abnormal ear
than in the normal ear. In sensorineural hearing loss, lateralization occurs to the normal
ear. The sensitivity of the test can be increased (up to 5 dB) by having the patient block
his or her external ear canals by simultaneously pressing the index fingers at the introit.
To perform the Rinne test, the vibrating tuning fork is placed over the mastoid region
until the sound is no longer heard. It is then held at the opening of the ear canal on the
same side. A patient with normal hearing should continue to hear the sound. In
conductive hearing loss, the patient does not continue to hear the sound, since bone
conduction in that case is better than air conduction. In sensorineural hearing loss, both
air conduction and bone conduction are decreased to a similar extent.
In the Schwabach test, the patient's hearing by bone conduction is compared with the
examiner's hearing by placing the vibrating tuning fork against the patient's mastoid
process and then to the examiner's. If the examiner can hear the sound after the patient
has stopped hearing it, then hearing loss is suspected.
The vestibular portion of the nerve enters the brainstem along with the cochlear portion.
It transmits information about linear and angular accelerations of the head from the
utricle, saccule, and semicircular canals of the membranous labyrinth to the vestibular
nucleus. Linear acceleration is monitored by the macules in the utricles and saccules;
angular acceleration is monitored by the cristae contained in the ampullae in the
semicircular canals. These signals reach the superior (Bechterew), lateral (Deiters),
medial (Schwalbe), and inferior (Roller) nuclei and project to the pontine gaze center
through the medial longitudinal fasciculus; to the cervical and upper thoracic levels of the
spinal cord through the medial vestibulospinal tract; to the cervical, thoracic, and
lumbosacral regions of the ipsilateral spinal cord through the lateral vestibulospinal tract;
and to the ipsilateral flocculonodular lobe, uvula, and fastigial nucleus of the cerebellum
through the vestibulocerebellar tract.
The Romberg test is performed to evaluate vestibular control of balance and movement.
When standing with feet placed together and eyes closed, the patient tends to fall toward
the side of vestibular hypofunction. When asked to take steps forward and backward, the
patient progressively deviates to the side of the lesion. Results of the Romberg test may
also be positive in patients with polyneuropathies, and diseases of the dorsal columns, but
these individuals do not fall consistently to 1 side as do patients with vestibular
dysfunction.
Another test is to ask the patient to touch the examiner's finger with the patient's hand
above the head. Consistent past pointing occurs to the side of the lesion. Provocative tests
include the Nylen-Bárány test and caloric testing (see Ancillary signs).
Glossopharyngeal nerve - CN IX
The nucleus of the nerve lies in the medulla and is anatomically indistinguishable from
the CN X and CN XI nuclei (nucleus ambiguous). Its main function is sensory
innervation of the posterior third of the tongue and the pharynx. It also innervates the
pharyngeal musculature, particularly the stylopharyngeus, in concert with the vagus
nerve.

13
Vascular stretch afferents from the aortic arch and carotid sinus, as well as chemoreceptor
signals from the latter, travel in the nerve of Herring to join the glossopharyngeal nerve;
they reach the nucleus solitarius, which in turn is connected to the dorsal motor nucleus
of the vagus and plays a part in the neural control of blood pressure.
Lesions affecting the glossopharyngeal nerve result in loss of taste in the posterior third
of the tongue and loss of pain and touch sensations in the same area, soft palate, and
pharyngeal walls. CN IX and CN X travel together, and their clinical testing is not
entirely separable. Therefore, examination of CN IX is discussed with that of the vagus
nerve.
Vagus nerve - CN X
Starting in the nucleus ambiguous, the vagus nerve has a long and tortuous course
providing motor supply to the pharyngeal muscles (except the stylopharyngeus and the
tensor veli palati), palatoglossus, and larynx. Somatic sensation is carried from the back
of the ear, the external auditory canal, and parts of the tympanic membrane, pharynx,
larynx, and the dura of the posterior fossa. It innervates the smooth muscles of the
tracheobronchial tree, esophagus, and GI tract up to the junction between the middle and
distal third of the transverse colon.
The vagus provides secretomotor fibers to the glands in the same region and inhibits the
sphincters of the upper GI tract. Along with visceral sensation from the same region, the
nerve participates in vasomotor regulation of blood pressure by carrying the fibers of the
stretch receptors and chemoreceptors (ie, aortic bodies) of the aorta and providing
parasympathetic innervation to the heart.
The pharyngeal gag reflex (ie, tongue retraction and elevation and constriction of the
pharyngeal musculature in response to touching the posterior wall of the pharynx,
tonsillar area, or base of the tongue) and the palatal reflex (ie, elevation of the soft palate
and ipsilateral deviation of the uvula on stimulation of the soft palate) are decreased in
paralysis of CN IX and CN X. In unilateral CN IX and CN X paralysis, touching these
areas results in deviation of the uvula to the normal side.
Unilateral paralysis of the recurrent laryngeal branch of CN X results in hoarseness of
voice. Bilateral paralysis results in stridor and requires immediate attention to prevent
aspiration and its attendant complications.
Spinal accessory nerve - CN XI
From the nucleus ambiguous, the spinal accessory nerve joins the vagus nerve in forming
the recurrent laryngeal nerve to innervate the intrinsic muscles of the larynx. The spinal
portion of the nerve arises from the motor nuclei in the upper 5 or 6 cervical segments,
enters the cranial cavity through the foramen magnum, and exits through the jugular
foramen, and provides motor innervation to the sternocleidomastoid (SCM) and the mid
and upper thirds of the trapezius.
In testing, functional symmetry of the SCM and the trapezius muscles should be
evaluated. Have the patient push the face against resistance to the right and to the left.
When the right SCM is weak, pushing to the opposite (ie, left) side is impaired, and vice
versa. Shrugging of the shoulder is impaired ipsilaterally when the trapezius is weak.
Hypoglossal nerve - CN XII
The nucleus of this nerve lies in the lower medulla, and the nerve itself leaves the cranial
cavity through the hypoglossal canal (anterior condylar foramen). It provides motor
innervation for all the extrinsic and intrinsic muscles of the tongue except the

14
palatoglossus. To test the hypoglossal nerve, have the patient protrude the tongue; when
paralyzed on 1 side, the tongue deviates to the side of paralysis on protrusion.
Examination of the Sensory and Motor Systems
Sensory system
Noncortical sensory system
This is constituted by the peripheral nerves with their central pathways to the thalamus.
Light touch, pain, heat, cold, and vibration sensations can be included in this group.
Light touch is tested by touching the skin with a wisp of cotton or tissue. Pain is tested by
using a sharp object such as an open safety pin. Temperature can be tested by touching
the patient's skin with 2 test tubes, 1 with warm water and the other with cold water.
Compare the 2 sides and also to a benchmark, such as the patient's own forehead
(assuming sensation there is normal). Vibration is tested with a tuning fork, preferably
with a frequency of 128 Hz. Compare findings on the 2 sides, and also compare findings
with those in the same body part of the examiner.
Cortical sensory system
The cortical sensory system includes the somatosensory cortex and its central
connections. This system enables the detection of the position and movement of the
extremities in space (ie, kinesthetic sensation), size and shape of objects (ie,
stereognosis), tactile sensations of written patterns on the skin (ie, graphesthesia), and
tactile localization and tactile discrimination on the same side or both sides of the body.
Position sensation is tested with the patient's eyes closed. The examiner moves various
joints, being sure to hold the body part in such a way that the patient may not recognize
movement simply from the direction in which the patient may feel the pressure from the
examiner's hand.
Stereognosis is tested by placing some familiar object (eg, ball, cube, coin) in the
patient's hand while his or her eyes are closed and asking the patient to identify the
object. Inability to recognize the size or shape is referred to as astereognosis.
Agraphesthesia is the inability to recognize letters or numbers written on the patient's
skin. These abilities are impaired in lesions of the right parietal region.
Motor system
Trophic state
Assess the 3 S s: size, shape, and symmetry of a muscle. Atrophy, hypertrophy, or
abnormal bulging or depression in a muscle is an important diagnostic finding in the
presence of different muscle diseases or abnormalities. Hypertrophy occurs with
commensurate strength from use and exercise; on the other hand, hypertrophy with
weakness is seen commonly in Duchenne muscular dystrophy. The shape may also be
altered when the muscle or tendon is ruptured.
Muscle tone
Muscle tone is the permanent state of partial contraction of a muscle and is assessed by
passive movement. The muscle may be hypotonic or hypertonic. Hypotonia is defined as
decreased tone and may be seen in lower motor neuron lesions, spinal shock, and some
cerebellar lesions. Hypertonia may manifest as spasticity or rigidity.
Pyramidal lesions result in spasticity that may manifest as a clasp-knife phenomenon (ie,
resistance to passive movement with sudden giving way, usually toward the completion
of joint flexion or extension). Bilateral frontal lobe lesions may result in paratonia or
gegenhalten (German for against-stop), in which resistance increases throughout flexion

15
and extension. Rigidity refers to increased tone associated with extrapyramidal lesions; it
may result in a cogwheel (stepwise) or lead-pipe (uniform) resistance to passive
movement.
Muscle strength
Use this muscle-strength scale when assessing and documenting muscle strength (Table
2).
Table 2. Muscle-Strength Scale
Score Description
0 Absent voluntary contraction
1 Feeble contractions that are unable to move a joint
2 Movement with gravity eliminated
3 Movement against gravity
4 Movement against partial resistance
5 Full strength
Involuntary movements
Involuntary movements include fibrillations, fasciculations, asterixis, tics, myoclonus,
dystonias, chorea, athetosis, hemiballismus, and seizures.
Fibrillations are not visible to the naked eye except possibly those in the tongue.
Fasciculations may be seen under the skin as quivering of the muscle. Although
fasciculations are typically benign (particularly when they occur in the calf), if
widespread, they can be associated with neuromuscular disease, including amyotrophic
lateral sclerosis (ALS).
Asterixis can be elicited by having the patient extend both arms with the wrists
dorsiflexed and palms facing forward and eyes closed. Brief jerky downward movements
of the wrist are considered a positive sign. Asterixis is commonly seen with metabolic
encephalopathies.
Tics are involuntary contractions of single muscles or groups of muscles that result in
stereotyped movements. Gilles de la Tourette syndrome can manifest with multiple tics
and elaborate, complex movements and vocalizations.
Myoclonus, as the word implies, is a muscle jerk; it is a brief (<0.25 seconds),
generalized body-jerk, which is sometimes asymmetric. These occur alone or in
association with various primarily generalized epilepsies.
Dystonias are muscle contractions that are more prolonged than myoclonus and result in
spasms. Examples include blepharospasm, spasmodic torticollis, oromandibular dystonia,
spasmodic dysphonia, and writer's cramp.
In athetosis, the spasms have a slow writhing character and occur along the long axis of
the limbs or the body itself; the patient may assume different and often peculiar postures.
The term chorea means dance. Quasi-purposeful movements affect multiple joints with a
distal preponderance.
Hemiballismus is a violent flinging movement of half of the body. It is associated with
lesions of the subthalamic nucleus (ie, body of Louis).
Seizures may result in orofacial or appendicular automatisms, repeated eye blinks, or
tonic or clonic motor activity.
Examination of Reflexes, Cerebellum, and Meninges

16
Reflexes
The different reflex responses may be grouped into 3 categories on the basis of their
clinical significance.
Primitive reflexes
These include the glabellar tap, rooting, snout, sucking, and palmomental reflexes. As a
rule, these signs are generally absent in adults. When present in the adult, these signs
signify diffuse cerebral damage, particularly of the frontal lobes (hence the term frontal-
lobe release signs).
Superficial reflexes
These are segmental reflex responses that indicate the integrity of cutaneous innervation
and the corresponding motor outflow. These include the corneal, conjunctival,
abdominal, cremasteric, anal wink, and plantar (Babinski) reflexes.
The corneal and conjunctival reflexes may be elicited by gently touching the appropriate
structure with a sterile wisp of cotton. The normal response is bilateral winking. Absence
of such a response implies CN V paralysis. Blinking of only 1 eye suggests weakness of
CN VII on the side that does not wink.
The abdominal reflex can be elicited by drawing a line away from the umbilicus along
the diagonals of the 4 abdominal quadrants. A normal reflex draws the umbilicus toward
the direction of the line that is drawn.
The cremasteric reflex is elicited by drawing a line along the medial thigh and watching
the movement of the scrotum in the male. A normal reflex results in elevation of the
ipsilateral testis.
The anal wink reflex is elicited by gently stroking the perianal skin with a safety pin. It
results in puckering of the rectal orifice owing to contraction of the corrugator-cutis-ani
muscle.
The best known of this group of reflexes is the plantar reflex. This reflex may be elicited
in several ways, each with a different eponym. The most commonly performed maneuver
is stroking the lateral aspect of the sole with a sharp object. The normal response is
plantar flexion of the great toe, which is considered an absent (negative) Babinski sign.
Dorsiflexion of the great toe (Babinski sign present) suggests an upper motor neuron
lesion and also is referred to as a positive Babinski sign. Dorsiflexion of the big toe also
may be associated with fanning out of the other toes, as detailed in Babinski's original
description, but most neurologists consider this an unnecessary accompaniment of an
abnormal response.
Flexion of the knee and hip may occur in the paretic leg with urinary and fecal
incontinence. This is referred to as the en-mass reflex. Lack of either response may
indicate absence of cutaneous innervation in the S1 segment or loss of motor innervation
in the L5 segment ipsilaterally.
Deep tendon reflexes
These are monosynaptic spinal segmental reflexes. When they are intact, integrity of the
following is confirmed: cutaneous innervation, motor supply, and cortical input to the
corresponding spinal segment.
These reflexes include the biceps, brachioradialis, triceps, patellar, and ankle jerks. The
musculocutaneous nerve supplies the biceps muscle. The radial nerve supplies the
brachioradialis and triceps. The femoral nerve supplies the quadriceps femoris, which
enables the knee jerk, and the tibial nerve supplies the gastrocnemius and the soleus.

17
Spinal roots that subserve these reflexes are listed below.
Table 3. Muscles and Spinal Roots
Muscle Spinal Roots
Biceps C5, 6
Brachioradialis C6
Triceps C7
Patellar L2-4
Achilles S4
On occasion, these root numbers are offset by 1 when the cervical and/or lumbosacral
plexuses are prefixed or postfixed.
Several systems for reflex grading exist. An example is provided below.
Table 4. Reflex-Grading System
Score Reflexes
0 Absent
1 Hypoactive or present only with reinforcement
2 Readily elicited with a normal response
3 Brisk with or without evidence of spread to the neighboring roots
4 Associated with a few beats of unsustained clonus
5 Sustained clonus
All textbooks now use a 0-4 scale to grade deep tendon reflexes, without a number
assigned for sustained clonus. The addition of the number 5 allows for easy
representation by using a stick figure. For a quick method of recording the reflex pattern,
see Media file 1.

Cerebellar signs
The cerebellum provides an important feedback loop for coordination of muscle activity
by integrating the functions of the cortex, basal ganglia, vestibular apparatus, and spinal
cord. Midline cerebellar dysfunction results in ataxia of gait, difficulty in maintenance of
upright posture, and truncal ataxia. Acute neocerebellar hemispheric lesions result in
additional signs.
The following are various cerebellar signs:
Ataxia, atonia, and asthenia
Intention tremor
Dyssynergia (incoordination)
Dysmetria
Dysrhythmia
Dysdiadochokinesis
Dysarthria (staccato or scanning speech)
Gait is tested by having the patient walk normally and in tandem. In the latter, the patient
is asked to walk with 1 foot immediately in front of the other (ie, heel to toe). A tendency
to sway or fall to 1 side indicates ataxia, suggesting ipsilateral cerebellar dysfunction.

18
Atonia and asthenia can occur in other lesions of the nervous system and are not specific
to the cerebellum; their testing is described elsewhere.
Intention tremor refers to an oscillating tremor that accelerates in pace on approaching
the target. Dyssynergia or incoordination results in loss of smoothness of execution of a
motor activity. Dysmetria results in overshooting or undershooting of a target while
attempting to reach an object. All 3 of these can be elicited by having the patient attempt
to touch alternately his or her nose and the examiner's finger.
Dysrhythmia refers to the inability to tap and keep a rhythm. It can be tested by tapping
the table with a hand (or the floor with a foot) and asking the patient to repeat the
maneuver. Dysdiadochokinesis is the inability to perform rapid alternating movements; it
can be tested by asking the patient to tap 1 hand on the other (or on the thigh) repeatedly
while simultaneously pronating and supinating the hand. Various combinations of the
above signs appear, depending on the extent and location of the lesion in the cerebellum.
Dysarthria is usually a sign of diffuse involvement of the cerebellum. It is characterized
by poor modulation of the volume and pitch of the speech, causing oscillations of these 2
qualities.

Meningeal signs
Signs of meningeal irritation indicate inflammation of the dura; these signs are described
below.
Nuchal rigidity or neck stiffness is tested by placing the examiner's hand under the
patient's head and gently trying to flex the neck. Undue resistance implies diffuse
irritation of the cervical nerve roots from meningeal inflammation.
The Brudzinski sign is flexion of both knees during the maneuver to test nuchal rigidity.
This indicates diffuse meningeal irritation in the spinal nerve roots.
The Kernig sign is elicited by flexing the hip and knee on 1 side while the patient is
supine, then extending the knee with the hip still flexed. Hamstring spasm results in pain
in the posterior thigh muscle and difficulty with knee extension. With severe meningeal
inflammation, the opposite knee may flex during the test (see Media file 2).
The Lasègue or straight-leg raising (SLR) sign is elicited by passively flexing the hip
with the knee straight while the patient is in the supine position. Limitation of flexion due
to hamstring spasm and/or pain indicates local irritation of the lower lumbar nerve roots.
Reverse SLR is elicited by passively hyperextending the hip with the knee straight while
the patient is in the prone position. Limitation of extension due to spasm and/or pain in
the anterior thigh muscles indicates local irritation of the upper lumbar-nerve roots.
System Survey and Ancillary Signs
System survey
Autonomic nervous system
Autonomic dysfunction results in abnormalities in the following: sweating, skin
temperature, cyanosis or pallor, trophic changes of skin or nails, and postural changes in
blood pressure. Observation (and any necessary additional testing) easily demonstrates
the presence or absence of these signs. Understanding these signs helps the examiner
assess the patient's neurologic condition.
Neurovascular system
The following may be tested by palpation of the pulses and use of appropriate
instruments:

19
Brachial plexus and bilateral blood pressures
Cranial and peripheral pulses
Arterial bruits
Neurocutaneous system
Several neurologic conditions have telltale cutaneous stigmata. Evaluation for the
following can provide valuable diagnostic clues: loss of skin pigmentation as in vitiligo,
white hair-lock in Vogt-Harada-Koyanagi disease, cutaneous tumors or ash-leaf spots in
tuberous sclerosis (see Media file 3), and cutaneous eruptions over a dermatome which
may signify herpes zoster (see Media file 4).
Coffee-brown pigmented (ie, caf é; au lait) spots of varying sizes, usually greater than 1.5
cm in diameter, and axillary freckling (see Media file 5) are seen in neurofibromatosis.
These are observed in addition to or in the absence of the characteristic blubbery
subcutaneous tumors that give the condition its name.
Tufts of hair (satyr's tail), dimples, and large moles along the spine may indicate spina
bifida occulta or diastematomyelia of the spinal column.
Skeletal system - Cranium, spine, bones, joints
Palpation of the skull can reveal congenital anomalies that may indicate underlying
abnormalities of the brain. In cephaloplegia, one half of the skull may be smaller than the
other, possibly signifying asymmetric brain development. Microcephaly or macrocephaly
may be detected by measuring the circumference of the head. Observation of the spine
may reveal the presence of myelomeningocele, scoliosis, and/or kyphosis. In cases of
prenatal brain injuries, the length of the long bones may be reduced on the side opposite
the cephaloplegia.
Trophic changes in the joints can be associated with denervation in tabes dorsalis or
Charcot-Marie-Tooth (CMT) disease. The distal muscular atrophy seen in CMT disease
gives the legs the appearance of inverted champagne bottles (see Media file 6). Muscular
atrophy seen in the region of the temporalis muscles and facial musculature associated
with frontal balding is typical of myotonic dystrophy (see Media file 7).
Pes cavus deformity (see Media file 8) can be associated with spina bifida and other
spinal dysraphisms. A young person with mental retardation, genu valgum, pes cavus,
and stroke may have homocystinuria, an inborn error of metabolism typically associated
with mental retardation (usually severe) and intimal thickening and necrosis of the media
of blood vessels, resulting in strokes and coronary artery disease.
Ancillary signs
Anisocoria
This refers to pupillary asymmetry, which may result from sympathetic or
parasympathetic dysfunction. Sympathetic dysfunction results in Horner syndrome, in
which the pupil is small but reacts to light. Hippus, a series of oscillating pupillary
contractions seen in response to light, is a benign condition. Argyll-Robertson pupil, seen
in neurosyphilis, is irregular and small; it does not react to light, but does accommodate.
In parasympathetic paralysis, the affected pupil is larger and reacts poorly or not at all to
light. Injury to the ciliary ganglion or short ciliary nerves results in a tonic pupil, which is
large and has slow or absent reaction to light. A benign form of tonic pupil is seen in
Adie syndrome, Holmes-Adie syndrome (ie, tonic pupil with absent patellar and Achilles
reflexes), and Ross syndrome (ie, tonic pupil with hyporeflexia and progressive
segmental hypohidrosis).

20
Anosognosia
This refers to denial of illness and typically is seen in patients with right frontoparietal
lesions, resulting in left hemiplegia that the patient denies. A form of visual anosognosia
(Anton syndrome) is seen in patients with bilateral occipital lobe infarctions; these
patients with double hemianopsia (bilateral cortical blindness) deny that they are blind.
Asterixis
This is seen in patients with metabolic encephalopathies. Momentary loss of tone and
flapping of the hand are seen when the patient extends his arms in front with the wrists
dorsiflexed.
Ataxia
Heel-to-toe tandem gait is tested by asking the patient to walk with 1 foot directly in front
of the other. Ataxia can be demonstrated in this manner.
Beevor sign
This is seen with bilateral lower abdominal paralysis that results in upward deviation of
the umbilicus when the patient tries to raise his head and sit up from the supine,
recumbent position.
Benediction hand
This is seen with lesions of the median nerve in the axilla and upper arm. When present,
the index finger remains straight and the middle finger partially flexes when the patient
tries to make a fist (assuming the position of the hand of a clergyman while saying the
benediction).
Bielschowsky sign
This refers to increasing separation of the images seen when a patient's head is tilted
toward the side of a superior oblique (trochlear nerve) paralysis. This sign by itself is not
diagnostic and should be used only as a supplement to other tests in suspected CN IV
paralysis.
Chvostek sign
This is seen in hypocalcemia. Tapping the cheek at the angle of the jaw precipitates
tetanic facial contractions.
Cogan sign
This is seen in myasthenia gravis. It refers to transient baring of the sclerae above the
cornea as the patient resumes the primary eye position after looking down.
Dalrymple sign
This refers to the upper-lid retraction seen in thyroid ophthalmopathy.
Doll's-eye maneuver
This refers to turning the head passively with the patient awake and fixated or when the
patient is in a coma. In the former, the eyes remain fixated at the original focus when all
gaze pathways are normal; in the latter, the eyes deviate in the opposite direction when
the brainstem is intact.
Gower sign
This sign, seen in severe myopathies, occurs when the patient attempts to stand up from
the floor. Patients first sit up, then assume a quadrupedic position, and then climb up their
own legs by using their arms to push themselves up.
Heterochromia iridis

21
This term refers to the difference in color of the 2 irides. It indicates early injury to the
sympathetic system. Ipsilateral to the injury the iris is blue or green, while the
contralateral iris is darker.
Jaw jerk
This is elicited by placing the examiner's index finger on the patient's lower jaw and then
striking it with the reflex hammer. An exaggerated reflex indicates the presence of a
pontine lesion. When the rest of the examination findings are normal, it may indicate
physiologic hyperreflexia.
Kayser-Fleischer ring
This is a brownish ring around the limbus of the cornea. It is best demonstrated during an
ophthalmologic slitlamp examination.
Lhermitte sign
This refers to the sensation of electricity associated with cervical spinal cord lesions
during passive or active flexion and extension of the neck. Once considered
pathognomonic of multiple sclerosis, it simply is the result of electricity generation by the
hypersensitive, demyelinated, or injured spinal cord; this sign can be associated with any
lesion in or around the cord.
Marcus-Gunn pupil
This sign requires a swinging-flashlight test to assess. As the flashlight swings from 1 eye
to the other, the abnormal pupil dilates as the light swings back from the normal side. No
anisocoria is seen. The phenomenon is also called a paradoxical pupillary reflex and
indicates an afferent (optic nerve) pupillary defect.
Milkmaid's grip
This refers to the inability to maintain a sustained grip commonly seen in patients with
chorea.
Moebius sign
This refers to weakness of ocular convergence (associated with proptosis) seen in
dysthyroid ophthalmopathy.
Myerson sign
Patients with Parkinson disease, particularly those with bilateral frontal lobe dysfunction,
continue to blink with repeated glabellar taps.
Nylen-Bárány sign
This is elicited by having the patient quickly lie down from the sitting position with the
head turned to 1 side and hanging down 30o below the horizontal over the edge of the
examining table. The procedure is then repeated with the head turned to the other side.
The test is positive when the patient experiences vertiginous discomfort and exhibits
nystagmus after a latency period of about 10 seconds. The nystagmus increases for about
10 seconds then fatigues in peripheral vestibular disease. In central lesions, nystagmus
may occur with the head turned to either side, without discomfort to the patient, and
without latency of onset or fatigue.
Ondine curse
This refers to the failure of autonomic control of breathing when the patient falls asleep.
Oommen sign
Have the patient close the eyes and place a pebble the size of an M&M candy on the palm
of the examiner's left hand. Cross the patient's middle finger over the index finger on its
dorsal aspect. With the examiner's right hand, hold the patient's crossed fingers and have

22
the patient's 2 (crossed) fingertips touch the pebble at the same time. Ask the patient how
many pebbles are in the examiner's hand. With normal stereognosis, the patient should
answer that there are 2 pebbles. In cases of astereognosis, the patient reports feeling only
1 pebble.
Opsoclonus
This refers to large-amplitude saccadic oscillations of the eyes in all directions, often
exacerbated by refixation. They persist during sleep and are associated with brainstem
and cerebellar lesions as well as a remote effect of certain carcinomas.
Optokinetic nystagmus
This is elicited by using a rotating, striped drum or a moving, striped piece of cloth. As
the patient's eyes fixate on a stripe, nystagmus seen in healthy individuals is due to the
optokinetic reflex. Lesions in the anterior aspects of the visual pathways decrease the
response, and lesions of the vestibular system result in a directional preponderance to the
elicited nystagmus.
Phalen sign
This refers to the aggravation of paresthesia and pain when the wrist is held in flexion (in
patients with carpal tunnel syndrome).
Roger sign
This is numbness of the chin in patients with lymphoreticular (and other types of)
malignancies.
Stellwag sign
This refers to decreased blinking frequency seen in thyroid ophthalmopathy.
Summerskill sign
This refers to the bilateral upper- and lower-lid retraction associated with severe liver
disease.
Tinel sign
This refers to the tingling sensation elicited by tapping along the path of a regenerating
nerve following injury. It helps to delineate the extent of nerve regeneration. The Tinel
sign also can be observed in tardy ulnar palsy (palpation at the elbow) and carpal tunnel
syndrome (tapping at the wrist).
Trendelenburg sign
This refers to the pelvic tilt toward the side of the unaffected raised leg when walking in
patients with lesions of the superior gluteal nerve.
Trombone tongue
This is seen in patients with chorea. It refers to the unsteadiness of the tongue when the
patient tries to protrude it outside the mouth.
Tullio phenomenon
This refers to the induction of vertigo and nystagmus with acoustic stimuli in patients
with labyrinthine disease.
von Graefe sign
This refers to the lid lag on down gaze in patients with thyroid ophthalmopathy.
Definition of Terms
Apoplexy - Stroke (see definition of Stroke)
Cataplexy - Sudden fall, usually due to loss of muscle tone; may be precipitated by
sudden changes in affect or mood in narcolepsy (see definition of Narcolepsy)
Cerebritis - Inflammation of the cerebral hemispheres

23
Encephalitis - Inflammation of the brain and brainstem structures
Encephalopathy - Dysfunction of the brain
Epilepsy - Recurrent seizures (see definition of Seizure)
Mononeuropathy - Dysfunction of individual nerves
Mononeuritis multiplex - Dysfunction of multiple single nerves
Myelitis - Inflammation of the spinal cord
Myelopathy - Dysfunction of the spinal cord
Myopathy - Primary muscle disease
Myositis - Inflammation of the muscles
Narcolepsy - Sudden attacks manifesting as an uncontrollable urge to sleep
Neuronopathy - Dysfunction of the cortical, cranial, or spinal neurons
Neuropathy - Dysfunction of the cranial or spinal nerves
Polyneuropathy - Bilateral symmetric ascending (stocking and glove) or descending
dysfunction of the peripheral nerves
Radiculopathy - Dysfunction of the nerve roots
Seizure - Subjective or objective behavioral manifestation of an abnormal and excessive
electrical discharge in the CNS
Stroke - Sudden onset of a neurological deficit, also known as a cerebrovascular accident

1. HEAD INJURY

Head injury is an exceptionally common form of injury world-wide.


Avoidance of head injury is an exceptionally difficult task to address in the short term but
obviously needs major attention.
An approach to the head injured should be one that addresses the avoidable complications
and recognizes these complications so that early treatment can be embarked upon. The
most important area of concern is the awareness of the pathophysiologic events that
follow the primary injury.
The primary injury is the immediate insult to the tissues of the head including the
intracranial contents. This primary insult could be focal or diffuse. The damage done in
the brain by the primary injury can for all intents and purposes not be undone. It has
been found that this primary injury very often is complicated by secondary injury. It is
the secondary injury that is avoidable. It is therefore vitally important to know about the
causes of secondary brain injury.
Secondary Brain Injury:
Systemic causes: hypotension, hypoxia, hypoglycaemia, anaemia, hypothermia,
hyperthermia, hypocapnea, hypercapnea, hyperglcaemia, acid-base disturbance,
electrolyte disturbance.
Intracranial cause: Mass lesion, cerebral swelling, cerebral blood flow alterations,
cerebral vasospasm, seizures, infection, hydrocephalus.
The aim of early treatment is therefore to prevent secondary damage by early detection of
the formation of mass lesion, by preventing damaging secondary events caused by
ischaemia, hypotension and seizures etc.
The management of head injury is heavily dependent in many cases on the availability of
specialized facilities.

24
There are vast distances that have to be covered to transfer critically injured patients in
this country. In many critically injured patients with head injury these factors all play
major roles in eventual outcome.
The Glasgow Coma Scale (GCS) is the only internationally recognized clinical grading
system and is used extensively in this country.
The Glasgow Coma Scale was developed in order to standardize the neurologic
assessment of patients with head injury. It was specifically designed to be easily
performed based upon clinical data, and to have a low rate of interobserver variability. In
addition, the Glasgow Coma Scale score is correlated with outcome in that patients with a
higher Glasgow Coma Scale score have a statistically better outcome than patients with a
lower Glasgow Coma Scale score.
The Glasgow Coma Scale score is determined by adding the values for eye opening,
verbal response, and motor response. Possible values range from 3 to 15. Note that
this scale rates the best response only. In patients who are intubated, in whom assessment
of best verbal response cannot be performed, notation of this is made in the Glasgow
Coma Scale score by adding a "t" to the end of the score. In patients who are intubated,
the best possible score would therefore be 11t. Certain numerical values of the Glasgow
Coma Scale have particular clinical significance. Patients with a Glasgow Coma Scale of
7 or less are considered to be comatose. Patients with a Glasgow Coma Scale score of 8
or less are considered to have suffered a severe head injury. Glasgow Coma Scale:
Best Eye Best Verbal
Points Opening Response Best Motor Response
6 - - Obeys
5 - Oriented Localizes pain
4 Spontaneous Confused Withdraws to pain
3 To speech Inappropriate Flexor (decorticate)
Extensor
2 To pain Incomprehensible
(decerebrate)
1 None None None

The following table illustrates the Glasgow Coma Scale (GCS): 3 clinical areas are
assessed.

Eye opening designated E. Verbal response designated V.


Motor response designated M.
E = 1 if there is no eye opening.
E = 2 if there is eye opening as a response of painful stimulus.
E = 3 if there is eye opening in response to the spoken word.
E = 4 spontaneous eye opening.

V = 5 oriented
V = 4 confused/disoriented
V = 3 inappropriate words

25
V = 2 incomprehensible
V = 1 no verbalization.

M = 6 obeys commands
M = 5 localizes stimulus
M = 4 withdraws from stimulus
M = 3 spastic flexion response (posturing)
M = 2 spastic extension response (posturing)
M = 1 no response.

By using the above the injuries can be graded into severity scale.

Severity category Glasgow Coma Sore


Minimal 15. No loss of consciousness or amnesia.
14. or 15 plus amnesia or
Mild brief (<5mins) LOC, or impaired alertness or
memory.
Moderate 9-13, or LOC>5mins., or focal neurologic deficit.
Severe 5-8
Critical 3-4
LOC = Loss of Consciousness.
With all the above in mind the following approach is suggested. Assess patient with the
use of GCS and also pupillary signs and other neurological signs.
1. Head injury – minimal (GCS = 15)
The injury site is treated appropriately. Symptomatic treatment is given. The patient can
be discharged with the advice of a good rest, at home, overnight. A head injury chart
could be very helpful.
2. Head injury – mild (GCS 14-15)
Treat the injury site.
In the case of an open wound a skull x-ray id helpful to exclude underlying fracture.
These cases should be observed for 24 hours or longer depending on progress. If in any
doubt regarding improvement or if deterioration supervenes a computerized tomography
(CT) of the brain should be obtained.
3. Head injury – moderate (GCS 9-13)
All must be admitted and observed neurologically. A CT scan of the brain must be
obtained. Skull x-rays are not necessary but cervical x-ray may be indicated depending
on the clinical evaluation. If on CT scan mass lesion is diagnosed the patient will have to
be sent to a neurosurgical unit.
If CT brain scanning facilities are not available and the patient has signs of papillary
inequality, localizing signs or if any doubt exists regarding urgency of the case, no time
should be wasted and the patient should be transferred to the applicable referral center.
Arrangements must be made with the trauma unit and the Neurosurgical doctor on call.
These cases have to be transferred with an adequate intravenous line and oxygen per face
mask.
Emergency room management
A quick thorough assessment is made.

26
A. Systemic survey: Vital signs.
Other injuries (cervical, chest, etc)
Mechanism of injury (include velocity, caliber, distance, homicide or suicide)
Baseline neurological state (always grade with Glasgow Coma Score)
Document the head wounds.
B. Resuscitation: This proceeds simultaneously with the above systemic survey.
Airway: In all cases of Glasgow Coma Score (GCS) < 8 intubate. If there is no
breathing problem allow patient to breath spontaneously via the tube and give oxygen per
tube. If there is apnea of hypocapnia ventilate. N.B. do not hyperventilate.
Normoventilation should be instituted.
Cardiovascular: prevent hypotension and hypovolaemia. Keep normovolaemic. If at
all possible place a central line for measuring central venous pressure and this also
provides a reliable line for fluid therapy.
Control Haemorrhage: external scalp haemorrhage is not a frequent problem with
compressive bandage.
C. Neurological Assessment:
GCS. Document in the standard way i.e. E. = -/4.,V. + -/5., M. = -/6.
Total score = -/15
Pupillary size.
Brainstem signs.
Localizing signs.
All patients should be loaded with an anticonvulsant e.g. Phenytoin Sodium (Epanutin)
10mg/Kg body weight (in adults a dose of 1 gm in 200ml of normal saline is infused over
30-60 minutes).
D. Radiologic Evaluation: If you are in peripheral hospital do not waste time refer to a
center with Neurosurgical facilities.
If in a hospital with neurosurgical facilities the patient should be sent for a brain CT and
cervical x-rays. A chest x-ray is of course mandatory if central line has been placed and
or if chest injury is suspected.
E. Salvageability: If GCS = 3-5 following adequate stabilization the prognosis is
usually poor. However, keep in mind the possibility of using cases as an organ donor and
treat appropriately.
Patients with a GCS = 6-8 may have a reasonable prognosis and should be referred for
neurosurgical assessment.
F. Other important issues: List all medication and times given. Provide telephone
numbers of relatives and if possible get relatives to sign consent for surgical procedures.
Give broad spectrum prophylactic antibiotic
Do not ventilate unless there is a specific reasons.
Indications for ventilation.
1. Respiratory indications other than of head injury.
2. Signs of pending cerebral herniation.
3. Hypercapnea.
4. Poor tolerance of endotracheal tube would need sedation. The sedation may well
cause further respiratory problems in the face of head injury and therefore it may be safer
to ventilate this patient. NB sedation can cause hypotension, keep eye on CVP.

27
If ventilation is required – do not hyperventilate but normoventilation is what should be
strived for.
Special Situations/Precautions.
1. Children. Refer all children with significant head injury and associated alteration in
level of consciousness to a neurosurgical unit. Special care and expertise is required
when intubating children. This is in fact a highly specialized task and should be
undertaken by an experienced critical care doctor or anesthetist. If there is no expertise in
intubation do not intubate but use nasopharyngeal tube/airway and supply oxygen per
mask. Position patient on side or semi prone. Take care of cervical spine and the patient
must be fitted with a cervical brace. Intravenous fluids in children have to be monitored
carefully. Maintain normal intake or monitor with CVP. Do not overhydrate, this can
cause rapid cerebral swelling and compromise outcome significantly. Know normal
haemodynamic parameters in children of all ages.
2. The intoxicated head injury patient. If these patients have a GCS < 8 they should be
referred for a CT scan.
3. The deteriorating head injury patient. The patient has a dilated pupil and altered level
of consciousness. Also may have localizing signs.
a. If distance is too far to referral center, do emergency burrholes and if mass lesion is
found e.g. extradural haematoma/subdural haematoma – proceed to craniectomy and
evacuate. Once patient is stable transfer to Neurosurgical or any critical care unit for
further evaluation and CT scan of brain. Do not hesitate to use lines of communication
for assistance.
b. If no facilities available (i.e. theatre facilities) contact nearest Neurosurgical/critical
care unit for advice.
c. If within 1 hour away from a specialized unit a case could be made out for using a
diuretic (Mannitol is drug of choice). Give 0.25 to 1 gram/kg body weight, of Mannitol
by intravenous route immediately. Make sure that patient is catherized. Make sure that
this patient is normal to slightly hypervolaemic.
Management of Concussion, Brain Contusion and Diffuse Axonal Injury.
Concussion.
Cerebral concussion is a diffuse brain injury thought to be caused by acceleration-
deceleration injury to the brain. Cerebral concussion is a spectrum of injuries, ranging
from mild to severe. Mild concussion is defined as no loss of consciousness with
transient neurologic disturbance. Moderate concussion is defined as loss of
consciousness with complete recovery occurring in less than 5 minutes. Severe cerebral
concussion is defined as unconsciousness lasting greater than 5 minutes. Evaluation and
treatment of patients with cerebral concussion remains controversial. Workup includes
complete history and physical examination, with neurological examination. Other tests
include cervical spine x-rays and other radiographs as indicated, blood alcohol level and
urine drug screen, and CT scan of the head in all patients except those who are
completely asymptomatic and neurologically normal.
Treatment of patients with cerebral concussion who have a Glasgow Coma Scale score
of 14 or 15 is usually expectant. Most patients should undergo hospital admission with
frequent neurological examinations. These include all patients with an abnormal CT
scan, history of loss of consciousness, decreased or decreasing level of consciousness,
severe headache, under the influence of alcohol or drugs, have physical examination

28
evidence of CSF rhinorrhea or otorrhea, significant associated injuries, no reliable
companion at home, unable to return promptly, or are amnestic for the injury. Only
those patients who do not manifest any of the prior criteria should be considered for
discharge from the hospital. If hospital discharge is considered, any of the previously
listed signs or symptoms should prompt a return to the hospital. A written head injury
"warning sheet" should be issued. Neurosurgical follow-up should be scheduled.
In addition to frequent neurologic examinations, patients admitted after cerebral
concussion may be treated with Tylenol or very mild doses of narcotic pain medication
for headache. Nausea and vomiting, which are frequently present after mild or severe
concussion, should be treated with non-phenothiazine antiemetic medication. Discharge
may be considered when the patient is neurologically normal, nausea and vomiting has
ceased, and headache has ceased or is adequately controlled.
All patients who have suffered a cerebral concussion should be counseled regarding the
possible occurrence of "post concussive syndrome". Prominent symptoms include
headache, mild impairment of memory, dysequilibrium, and alteration of mood.
These symptoms usually regress spontaneously but may persist for weeks to months.
Diffuse axonal injury.
Diffuse axonal injury is the most severe form of diffuse brain injury. It is felt to be the
most common cause of prolonged posttraumatic coma that is not due to mass lesion or
ischemia. Diffuse axonal injury is characterized by focal hemorrhagic lesions involving
the corpus callosum, rostral mid brain, superior cerebellar peduncles combined with
microscopic evidence of widespread axonal damage. Patients with diffuse axonal
injury frequently manifest decorticate or decerebrate posture and autonomic
dysfunction in addition to their prolonged coma. Elevated intracranial pressure is
frequently absent. Care is primarily supportive. In patients with prolonged coma, the
prognosis is generally poor, with a 50% mortality and with an approximately 25%
incidence of favorable outcome.
Cerebral Contusion.
A cerebral contusion is a focal brain injury caused primarily by impact of the brain
surface and the bony ridges of the calvarium. Cerebral contusions are frequently found in
the region of the frontal poles, anterior skull base, adjacent the sphenoid ridge, and at the
temporal poles. Other locations include the cerebellar hemispheres and the occipital
poles. A characteristic pattern of cerebral contusion called the "coup and contrecoup"
injury is frequently seen. The coup contusion occurs at the sight of impact and the
contrecoup contusion occurs in the brain at the point diametrically opposite the point of
impact.
Treatment of cerebral contusion is guided by the neurologic examination. The patient
should be admitted to the hospital for observation and frequent neurologic examinations.
Intracranial pressure monitoring and treatment should be instituted for the comatose
patient. While surgical treatment, consisting of debridement of the contused brain tissue,
is not routinely recommended, it can be considered in patients with refractory intracranial
hypertension.
Management of Acute Subdural and Epidural Hematoma.
Subdural hematoma.
In the acute traumatic subdural hematoma, blood collects between the dura mater and
surface of the brain. Most commonly, the bleeding results from tearing of bridging veins

29
located over the convexity of the brain surface. Bleeding originating from a small cortical
artery represents the second most common source. Associated intracranial lesions,
particularly cerebral contusions, are found in at least 50% of patients with acute traumatic
subdural hematoma.
Patients presenting with acute traumatic subdural hematoma may range from normal to
deeply comatose. Unlike the epidural hematoma, to be described later, the most common
presentation of the patient with an acute traumatic subdural hematoma is that of a patient
rendered unconscious at the time of injury without regaining consciousness prior to
presentation.
Neurologic findings may be secondary to mass effect, elevated intracranial pressure,
and/or associated brain injuries. Elevated intracranial pressure is a common finding and
evaluation and treatment of elevated intracranial pressure, as outlined above, should be
instituted immediately. The acute traumatic subdural hematoma is most commonly
treated surgically. Observation should only be considered in those patients with small
(less than 10 mm thick) subdural hematomas who are neurologically intact. Because the
acute traumatic subdural hematoma consists of solid blood clot, burr hole drainage is
inadequate for relief of mass effect. A craniotomy should be performed and all easily
assessable blood clots should be removed. Postoperatively, patients frequently manifest
intracranial hypertension, and this should be treated.
Outcome after treatment of acute traumatic subdural hematoma is related to a number of
factors, particularly preoperative neurologic status. Mortality ranges from greater than
75% in those patients who present with a GCS of 3 to 5 to minimal in patients who
present with GCS of 12 to 15. Time from injury to surgical decompression, elevated
intracranial pressure, and associated brain lesions also have a detrimental effect on
outcome.
Epidural hematoma.
In the acute epidural hematoma, blood collects between the inner surface of the calvarium
and the dura mater. Most commonly, the acute epidural hematoma results from fracture
of the skull, stripping the dura mater from the inner table of the skull, and causing
laceration of meningeal vessels or dural sinuses. Bleeding from the middle meningeal
artery is responsible for many supratentorial epidural hematomas. In contrast to the
subdural hematoma, the patient harboring an acute epidural hematoma may have an
initial loss of consciousness, followed by a brief "lucid" interval, followed by progressive
neurologic decline. This presentation is seen in approximately 1/3 of patients having an
acute epidural hematoma.
Treatment of acute epidural hematoma is generally surgical. Patients with small epidural
hematomas not traversing a meningeal artery vein or major sinus, who present greater
than 6 hours after injury may be considered for a nonoperative therapy. However,
admission with frequent neurologic examination and a low threshold for repeat CT
scanning is mandatory. For all other patients, operative treatment is recommended. As
with the acute subdural hematoma, the acute epidural hematoma is comprised of a solid
blood clot. Therefore, burr hole drainage is inadequate for removing the intracranial
mass. A craniotomy should be performed and the entire blood clot evacuated.
Postoperatively, the patient is monitored for signs of elevated intracranial pressure.
Elevated intracranial pressure, if detected, is treated.
As in the acute traumatic subdural hematoma, outcome after treatment of acute epidural

30
hematoma is related to a variety of factors, the most important being preoperative
neurologic status. Mortality ranges from less than 15% in those patients who present with
GCS of less than 8 to very low in patients who present with GCS 8 to 15.
Management of Elevated Intracranial Pressure in Head Trauma:
Brain injury after acute head trauma can be divided into two categories. The first
category is primary injury, which is suffered at the time of impact. The second category
is secondary injury, which may occur at any time from that point forward. One of the
most important causes of secondary brain injury in head trauma is felt to be elevated
intracranial pressure (ICP).
In treatment of the patient with head trauma, the possibility of elevated intracranial
pressure should always be considered. Management of the traumatized patient begins
with the primary survey and resuscitation.
Airway patency with cervical spine control: It is important to establish the presence of
a patent airway. If such an airway is not present, one should be established. This may
include the use of chin lift or jaw thrust, clearance of foreign bodies, endotracheal
intubation, or creation of a surgical airway. It is important to consider that the cervical
spine may be injured and that it should be maintained in a neutral position during any of
the above maneuvers.
Breathing control. The chest should be examined and the rate and depth respiration
determined. Inadequacy may indicate the need for mechanical ventilation. High
concentrations of oxygen should be administered. Pneumothorax should be treated.
Circulatory and hemorrhage control. The quality, rate, and regularity of the pulse
should be determined. Sights of major hemorrhage should be identified and treated.
Disability. A brief neurologic examination should be performed. The Glasgow Coma
Scale should be determined. Pupils should be assessed for size, equality, and reaction.
During the secondary survey, a more complete neurologic examination should be
performed including evaluation of the patient’s strength, sensation, reflexes, and
remaining cranial nerves.
Following the secondary survey, appropriate imaging studies should be obtained. In a
patient with obvious craniofacial trauma, mechanism of injury sufficient to produce brain
injury, or a disturbed level of consciousness, a CT scan of the head without contrast
should be performed. The presence of fractures, foreign bodies, space occupying lesions,
or hemorrhage should be noted, as well as the ventricular size.
Intracranial pressure monitoring should be considered in the following situations:
Patients with an abnormal admission CT scan and Glasgow Coma Scale score of 3 to
8 after cardiopulmonary resuscitation, or, patients with a normal head CT with a Glasgow
Coma Scale score of 3 to 8 and the presence of two or more of the following features:
Age over 40 years, unilateral or bilateral motor posturing, systolic blood pressure
less than 90 mmHg. The current preferred modality for monitoring of intracranial
pressure is the placement of a ventricular catheter (ventriculostomy). Use of fiberoptic
or strain gauge pressure monitors can be considered.
Elevated intracranial pressure has been shown to have definite prognostic implications in
a patient with severe brain injury. In addition, it is generally held that treatment of
elevated intracranial pressure may improve outcome in the patient with severe brain
injury. The currently recommended threshold for treatment of elevated intracranial
pressure is 20 to 25 mmHg. Interpretation and treatment of intracranial pressure based on

31
this threshold value should be corroborated by frequent clinical examination and
assessment of the cerebral perfusion pressure. Current recommendations suggest that
CPP should be a minimum of 60 to 70 mm of mercury. It is important to consider that,
while MAP is an important determining factor in the CPP, it has also been shown that
low MAP is an independent predictor of poor outcome.
After elevated intracranial pressure has been identified, treatment should be initiated. In
general, treatment should proceed in a stepwise fashion, beginning with the least onerous
treatment modalities. Escalation of treatment should proceed only after failure of less
onerous modalities. The suggested hierarchy of treatment includes the following
therapeutic modalities:
Body positioning. The head should be elevated 30 degrees. The neck should be
maintained in a neutral position. Compression of the jugular veins should be avoided.
Maintenance of homeostasis. Euvolemia should be established. Arterial blood gases
should be measured with the goal of maintaining the PO2 in the 90 to 100-mm mercury
range and the PCO2 in the 35 to 40-mm mercury range.
Mild sedation. This is most frequently carried out using a combination of
benzodiazepines and/or narcotics.
External ventricular drainage. At this stage, placement of a ventriculostomy, if not
done previously for ICP measurement, should be considered. The reservoir is generally
placed 5 to 10 cm above ear level or, alternatively, placed at ear level and opened at
regular intervals.
Use of osmotic diuretics. Mannitol is the most commonly used osmotic diuretic. It is
most frequently given in a dose of 0.25 to 1.0 grams/kg I.V. over 15 minutes. If the effect
of treatment with Mannitol is transient, the dose may be repeated, so long as the serum
osmolality remains less than or equal to 320 mOsm/L and the patient remains euvolemic.
Moderate hyperventilation. At this stage, moderate hyperventilation to a PCO2 of 30 to
35 mm of mercury can be considered.
Second tier therapies including barbiturate therapy. At this stage, barbiturate therapy
can be considered. Pentobarbital is the most commonly used barbiturate for the treatment
of refractory intracranial hypertension. Recommended loading dose is 10 mg/kg over 30
minutes followed by a maintenance dose of 1 mg/kg per hour as a continuous infusion.
The dose is then titrated to achieve serum Pentobarbital levels in the range of 3 to 4 mg/dl
or an electroencephalographic pattern of burst suppression. Potential complications of
this modality of treatment are numerous, with hypotension being the primarily dose-
limiting toxicity. Barbiturate treatment of refractory intracranial hypertension has been
shown to decrease mortality but has not been shown to improve neurologic outcome.
The above treatment algorithm assumes that all significant cranial space-occupying
lesions have been appropriately surgically treated. During implementation of the above
algorithm, it is important to consider the possibility that a new intracranial lesion has
developed. Because of this, a repeat CT scan of the head should be considered prior to
escalation of therapy.
Note that the above treatment algorithm does not include the use of corticosteroids. The
use of glucocorticoids is not recommended for improving outcome or reducing
intracranial pressure in patients with severe head injury. The routine use of prophylactic
anticonvulsant medication is not recommended for the prevention of posttraumatic
seizures in the patient without a premorbid seizure disorder. The use of anticonvulsants

32
may be considered, in the first 7 days after injury, to prevent early posttraumatic seizures
in patients who are at high risk. Phenytoin and carbamazepine are the most commonly
used agents. Finally, nutritional support of brain injured patients should be instituted
within 7 days of injury.
Anti epileptics
Load all of the following patients with Phenytoin (Diphenylhydantoin/Epanutin)
10mg-20mg per Kg as IV infusion. Usually in adults we give 1 gram in 200ml of Saline
(NB – Phenytoin is not compatible with any glucose containing fluid) over ½ hour – 1
hour.
In children <2 years – Phenobarbitone 10mg per Kg as slow IVI injection should be
given.

Indications for anti epileptics in acute head trauma:


1. Severe head injury – GCS < 8.
2. Head injury with localizing signs.
3. In-driven skull fracture.
4. Any case where an intracranial mass lesion has been diagnosed.
5. Any case of intracerebral contusion.
Brain Herniation Syndromes in Trauma:
Distortion of the midline brain structures secondary to brain trauma may lead to specific
combinations of signs and symptoms which are collectively referred to as herniation
syndromes. In general, these symptoms result from the distortion of midline brain
structures secondary to brain swelling, hydrocephalus, or intracranial mass lesions. While
numerous herniation symptoms have been described, the two most commonly seen
syndromes in the setting of trauma are uncal herniation and tonsillar herniation.
Uncal herniation:
Most often results from a laterally placed mass displacing the brain stem contralaterally
and pushing the uncus of the temporal lobe medially over the tentorial edge.
Early Sign: Ipsilateral pupillary dilation
Late Signs: Complete ipsilateral third nerve palsy,loss of consciousness,contralateral
hemiplegia (secondary to mass), ipsilateral hemiplegia (secondary to compression of
contralateral cerebral peduncle against edge of tentorium (Kernohan’s notch), flaccid
paralysis’
Tonsillar herniation:
Results from downward displacement of the cerebellar tonsils through the foramen
magnum, causing compression of the cervicomedullary junction. Frequently secondary to
posterior fossa mass. May be precipitated by lumbar puncture in the presence of such a
mass.
Signs: Head tilt/neck pain, respiratory arrest, loss of consciousness, flaccid paralysis.
PENETRATING MISSLE INJURY TO THE HEAD
Pathology
Can be defined into: high and low velocity injuries. For practical purposes high velocity
injuries to the head are usually fatal.
Factors which influence the severity of injury are: Muzzle velocity, distance of flight,
caliber, trajectory through cranium, eloquence of damage brain, vascular injury, and
subsequent complications.

33
Primary injury is the injury caused by the impact. The damage done by the primary
insult cannot be reversed. The direct impact causes crushing and laceration of tissue as
the missile penetrates skin, skull and brain. Bits of fragmented bone can act as secondary
missiles and cause more widespread damage. High-speed shockwaves are generated
when missile enters a tissue medium and causes a pulsating temporary cavity. The size
of the cavity can be may times the size of the missile. The cavity essentially forms a
tract, which contains blood clot, in-driven debris, necrotic and contused brain tissue. The
pulsations of the cavity generate pressure waves, which radiate to distant locations.
Secondary injury is caused by a number of systemic disturbances which may accompany
severe head injury. These are hypotension, hypoxia, hypo/hyper-glycaemia,
hypo/hyper-capnia. There are also a number of secondary intracranial events that add to
the primary injury. These include intracranial haematoma, raised intracranial
pressure, seizures to name a few.
It is quite clear that the primary injury cannot be reversed but secondary injuries can be
prevented, and some diagnosed and treated early to prevent the effects of some of the
secondary events.

Penetrating Trauma.
Penetrating injuries include all injuries where the scalp and skull are violated by foreign
objects including knives, sticks, pencils, arrows, and bullets. Low velocity injuries, such
as those produced by a knife blade, produce brain injury along the tract of the knife. High
velocity injuries, such as those produced by a bullet, produce both local injury along the
tract of the bullet, as well as remote injury in the cavity produced in the wake of the
bullet.
The size of the cavity produced by a bullet is related to its kinetic energy, as well as its
shape. The kinetic energy is proportional to the mass of the bullet as well as the square of
its velocity. The shape of the bullet not only affects the velocity, but also its ability to
transfer its kinetic energy to the brain tissue. Bullets that tumble or deform on impact
transfer a greater proportion of their kinetic energy to brain tissue and thus produce a
more severe injury.
After resuscitation and primary survey, an evaluation of the patient with a penetrating
injury includes a thorough physical examination and neurological examination. The
location of entry and exit wounds should be well documented. Extent of tissue loss
should likewise be documented. All patients who have had penetrating brain injury
should undergo a CT scan of the head. Coronal CT scan of the head should be considered
in patients with involvement of the anterior skull base. Cerebral angiography should be
considered in patients where vascular injury is suspected. Cerebral angiography should be
given particular consideration where the tract of the injury passes close to a major
vascular structure or in patients with a significant subarachnoid hemorrhage or delayed
hematoma.
As in patients with severe non-penetrating brain injury, elevated intracranial pressure
should be suspected. The algorithm for this is described above. In addition, in patients
with penetrating brain injury, intracranial pressure monitoring is indicated when it is not
possible to assess the neurological examination accurately or when the need to evacuate a
mass lesion is unclear.

34
As with the open depressed skull fracture, most penetrating brain injuries require surgical
treatment.
Entrance and exit wounds. Treatment of small bullet entrance wounds where the scalp is
not devitalized and the patient has no significant intracranial pathology may be treated
with local wound care and closure. More extensive wounds with nonviable scalp, bone,
or dura may require operative debridement before primary closure and dural grafting.
Watertight dural closure is recommended. This includes patients with significant
fragmentation of the skull.
Intraparenchymal lesions. Intraparenchymal lesions resulting in significant mass effect
may require debridement of necrotic brain tissue and removal of easily accessible bone
fragments. This includes patients with significant intracranial hematomas.
Injuries resulting in communication between an open-air sinus and the intracranial space
should be closed in a watertight fashion.
Routine removal of bone fragments or missile fragments remote from the entry site is not
recommended. Vascular injuries detected on arteriography may require surgical or
endovascular treatment as indicated. Cerebrospinal fluid leaks which do not close
spontaneously or which do not resolve after the primary surgery should be treated with
temporary CSF diversion. Those which do not respond to temporary CSF diversion may
require surgical treatment. Patients with penetrating brain injury should be administered
broad-spectrum antibiotics prophylactically. The duration of this treatment is somewhat
controversial. Finally, the use of anti-seizure prophylaxis in the first week of penetrating
brain injury is recommended to prevent early posttraumatic seizures. As with severe non-
penetrating brain injury, long-term prophylactic treatment with anticonvulsants to prevent
late posttraumatic seizures is not recommended.
Principles of Management of Open, Closed, and Basilar Skull Fractures.
Open skull fractures.
The treatment of skull fractures with overlying laceration is primarily based on whether
the fracture is depressed or non-depressed. Open non-depressed fractures may be treated
with inspection, cleansing, and scalp suturing with an acceptably low rate of infection.
Open depressed skull fractures present a significant risk of infection. They are generally
treated with operative irrigation, debridement, and removal of the depressed fragments.
Frequently, this will necessitate further surgical procedures to correct the resulting
cosmetic deformity.
Closed fractures.
Closed skull fractures require no specific treatment. As noted above, the patient harboring
a skull fracture is at increased risk of formation of epidural hematoma. Patients harboring
this injury should be treated as for those patients with severe concussion, unless
symptoms of elevated intracranial pressure develop. Traditionally, closed depressed skull
fractures were treated surgically to elevate the depressed fragments. It has since been
shown that this practice does not result in a decreased incidence of posttraumatic epilepsy
and has therefore been abandoned. Treatment of patients with depressed, closed fractures,
except where there is a significant cosmetic deformity, should be treated as outlined
above for closed, non-depressed fractures.
Basilar skull fractures.
A basilar skull fracture involves the cranial base. The most common sites are the floor of
anterior cranial fossa and the temporal bone. Evidence that a patient may have suffered a

35
basilar skull fracture includes "raccoon eyes" and "battle sign" (bruising posterior to the
ear, possibly including the mastoid process). Most basilar skull fractures do not require
treatment in the absence of associated brain injury. They are a sign that a significant blow
has been delivered to the cranium. Most patients with significant basilar skull fracture
should be observed with frequent neurological examinations for 24 hours after the injury.
Occasionally, complications of basilar skull fracture may result. These include
posttraumatic cerebrospinal fluid leakage, optic nerve injury, and facial nerve injury. The
incidence of posttraumatic cerebrospinal fluid leak after a closed head injury is
approximately 2%. The most common sites of cerebrospinal fluid egress are the nose
(CSF rhinorrhea) and ear (CSF otorrhea). Most traumatic CSF leaks resolve with
nonoperative treatment including head elevation. It is important to insure that the CSF
leakage does resolve, as persistent CSF rhinorrhea carries with it an approximately 25%
risk of meningitis, as well as a risk of tension pneumocephalus.
Posttraumatic CSF leak.
Treatment of the posttraumatic CSF leak which does not resolve with head elevation
includes repeated high volume lumbar punctures, as well as continuous catheter CSF
drainage. There is a finite risk of inducing tension pneumocephalus using those
modalities. For leaks which persist despite these measures, surgical treatment should be
considered.
Wound Care
Patients who have lacerations, abrasions and cuts of the scalp have appropriate standard
wound care. Sepsis is associated with poor primary wound care. All debris in the wound
should be meticulously removed and washed out with saline or antiseptic. In cases of
open in-driven skull fracture with dural penetration the wounds should be primanly
washed out with N Saline and debrided locally. Do not remove bone fragments. Refer
the patients for CT scan and to neurosurgical center.
MANAGEMENT OF CHRONIC SUBDURAL HEMATOMA.
As described above, the chronic subdural hematoma is a collection of blood and blood
products between the inner surface of the dura mater and the outer surface of the brain.
Unlike the acute traumatic subdural hematoma, onset of symptoms and detection of the
subdural hematoma occurs much later in the course. The hallmark of the chronic subdural
hematoma is blood products, visualized on the CT scan, which are isodense or
hypointense with respect to brain tissue. It is theorized that, like the acute traumatic
subdural hematoma, the source of the blood products is lacerated bridging veins resulting
from acceleration-deceleration forces applied to the skull. While this is usually the result
of trauma, trauma may be mild, remote, and not remembered by the patient or family.
Many patients who present with a chromic subdural hematoma have one or more risk
factors. These risk factors include; advanced age and cerebral atrophy, male sex,
coagulopathy. intracranial hypotension secondary to CSF shunting procedures, chronic
alcoholism.
Chronic subdural hematomas tend to enlarge slowly over time. The most common
mechanism of enlargement appears to be multiple episodes of re-bleeding, however,
other mechanisms may also be important.
Symptoms at presentation include symptoms of increased intracranial pressure (headache,
papilledema, decreased level of consciousness), as well as those of hemispheric mass
effect (hemiparesis, dysphasia, tremors, and dystonia. Seizures occur occasionally.

36
Treatment of chronic subdural hematoma is generally surgical. While treatment with
prolonged bedrest, head elevation, and osmotic diuretics has produced acceptable results,
the risks of prolonged immobilization in these generally debilitated patients frequently
outweigh the benefits.
There are multiple surgical options for the treatment of chronic subdural hematoma
including; twist drill hole and drainage, multiple burr holes and drainage, craniotomy and
drainage with stripping of membranes.
Hematomas that are not fully liquefied may require craniotomy. In addition, recurrence
rate after evacuation of chronic subdural hematoma is substantial.
Postoperatively, elevated intracranial pressure is unusual. Patients are generally
maintained supine with minimal elevation of the head for at least 24 hours to allow some
re-expansion of the brain. Because of the substantial rate of recurrence, neurosurgical
follow-up is essential.
Chronic subdural hematoma in children.
Like the adult chronic subdural hematoma, blood and blood products, in the childhood
chronic subdural hematoma, accumulate between the inner surface of the dura and the
brain surface. In children, it is particularly important, and sometimes difficult, to
distinguish between the chronic subdural hematoma and the subdural hygroma, in which
cerebrospinal fluid collects between the dura and arachnoid membrane. Furthermore,
low-density fluid may collect between the arachnoid and pia mater secondary to
communicating hydrocephalus.
While trauma is the most common cause of chronic subdural hematoma in children, the
possibility of non-accidental trauma (child abuse) must always be considered, particularly
in children less than 2 years of age. Coagulopathy remains a major risk factor.
In children, presenting symptoms are usually those of elevated intracranial pressure
including vomiting, lethargy, irritability, or increase in the head size. Seizures are also
seen.
The treatment of chronic subdural hematoma in children is different than that of adults.
Unlike adult patients, very young patients may have an open fontanel, facilitating
percutaneous aspiration of the subdural hematoma. Failure of this treatment modality
generally requires placement of a subdural to peritoneal shunt.
MANAGEMENT OF PAEDIATRIC TRAUMATIC BRAIN INJURY
PATHOPHYSIOLOGY
The disproportionately larger and heavier head and weak neck muscles of children render
them particularly prone to head injury after trauma. The primary cause of head injury
varies with age. In infants, non-accidental injury should be considered; toddlers
frequently suffer falls, whereas road traffic accidents and sports related injuries are more
common in older children and adolescents.
Primary Brain Injury
Occurs at the time of in initial injury, and may result in brain contusion, laceration, and
haematoma formation or diffuse axonal injury. Younger children are more likely to
develop subdural haematomas and diffuse cerebral oedema without a skull fracture,
whereas in adolescents, skull fractures, contusions and extradural haematomas are more
common.
Secondary Brain Injury

37
Occurs in the minutes to days after the initial injury and may be due to hypotension,
raised intracranial pressure or cerebral ischaemia. Secondary brain injury is worsened by
hypoxia, hypercarbia, anaemia, pyrexia, hypoglycaemia or hyperglycaemia. Secondary
brain injury may be modified by simple clinical interventions, the most important of
which are avoidance of hypotension and hypoxia.
Intracranial pressure
The brain is enclosed within the rigid skull. The intracranial pressure (ICP) is determined
by the volume of the brain tissue, the cerebrospinal fluid (CSF) volume, and the cerebral
blood volume. Increase in the volume of the intracranial contents, for instance due to
cerebral oedema or an extradural, subdural or intracerebral haematoma, will result in
increased intracranial pressure. Compensation is possible for small rises in ICP by
increased CSF absorption and a reduction in intracranial blood volume. Infants can
compensate for slow increases in intracranial pressure because of their open fontanelles
and suture lines; however sudden acute changes in intracranial pressure are not well
tolerated at any age. If compensatory mechanisms are overwhelmed, intracranial pressure
will increase rapidly and the brain will herniate through the structures within the skull or
the foramen magnum (‘coning’) to cause coma and death.
Cerebral perfusion pressure
The cerebral perfusion pressure (CPP) is the effective blood pressure that perfuses the
brain and is defined as:
CPP = MAP – ICP
where MAP is the mean arterial pressure. The MAP and therefore CPP vary with age, as
does the ICP. Table 1 shows the normal systolic BP in children, also the optimal level
systolic BP to be achieved in children with traumatic brain injury, that is, >95th centile for
age. Cautious fluid resuscitation and infusion of vasoconstrictors such as noradrenaline
may be required.
Table 1. Normal systolic blood pressure and optimal systolic blood pressure in children
with traumatic brain injury.
Age Normal systolic BP Optimal systolic BP in traumatic
brain injury
(mmHg)
(mmHg)
<1 year 70-90 >100
1-5 years 80-100 >95
6-11 years 90-110 >105
>12 years 100-120 >120
Typical intracranial pressures are 8–18mmHg in adults and 2–10 mmHg in children. The
venous pressure is low under normal circumstances and does not affect the CPP, but
abnormally raised venous pressure will reduce CPP.
Cerebral blood flow
Under normal conditions, cerebral blood flow is maintained at a constant level to meet
the metabolic demands of the brain over a wide range of MAP by the process of
autoregulation. The autoregulation range for adults is 50-140 mmHg. The autoregulation
range is not known in infants and children, but is likely to be around 40-90 mmHg.
Cerebral autoregulation is impaired by acute brain injury; in this situation, cerebral blood
flow follows cerebral perfusion pressure passively. It is vital to keep the blood pressure

38
within the ‘high normal’ range in head injured patients, as hypotension will result in
cerebral ischaemia.
In the absence of invasive measurement of intracranial pressure, cerebral perfusion
pressure should be maintained between 50-70 mmHg, that is, MAP 70-90 mmHg,
assuming the intracranial pressure to be 20 mmHg. The MAP may be calculated from:
systolic/diastolic BP: MAP = DBP + (SBP-DBP/3).
Cerebral perfusion pressure less than 50 mmHg has been demonstrated to be a predictor
of poor outcome in severe traumatic brain injury in adults and children. Extreme
hypertension will result in increased cerebral blood flow and cerebral oedema and should
also be avoided.
OTHER FACTORS AFFECTING CEREBRAL BLOOD FLOW AND INTRA
CRANIAL PRESSURE
Oxygen
An adequate supply of oxygen is essential to meet the metabolic requirements of the
brain. In addition, hypoxia results in cerebral vasodilatation and increase in cerebral
blood flow and hence intracranial pressure. Cerebral blood flow begins to rise when PaO2
falls to 6.7kPa (50mmHg), and doubles at PaO2 of 4kPa (30 mmHg). Hypoxia must be
avoided at all times in patients with head injury.
Carbon dioxide retention
Is a potent cause for cerebral vasodilatation and increase in intracranial pressure.
Conversely, hyperventilation resulting in hypocarbia results in cerebral vasoconstriction
and at extreme levels will cause cerebral ischaemia. It is important to maintain the carbon
dioxide levels within normal range as much as possible (4.5-5.5 kPa). Remember, end
tidal CO2 measurements usually underestimate arterial CO2, especially if there is
coexisting lung injury or disease. Modest hyperventilation may be used to reduce
intracranial pressure in an emergency (but keep PaCO2 > 4KPa, and definitely not below
3.5).
Body temperature
Has an important effect on cerebral blood flow. For every 1°C increase in body
temperature, there is a 5% increase in cerebral metabolic rate leading to an increase in
cerebral blood flow and intracranial pressure. Pyrexia should be avoided in the head
injured patient, and normothermia/moderate hypothermia is desirable. Excessive
hypothermia therapy below 33°C may increase mortality.
Blood glucose
The brain is critically dependent on a normal blood supply of glucose for metabolism and
only has a very small store of glucose in the form of glycogen. Children are more
susceptible to hypoglycaemia as the cerebral metabolic rate is higher in children than
adults (peak at 6 years). Conversely, hyperglycaemia should be avoided after head injury
as it induces lactic acidosis and production of oxygen free radicals and worsens
outcomes. It is important therefore to actively seek and treat hypoglycaemia and
hyperglycaemia.
Seizures
Seizures increase the metabolic demands (and therefore oxygen requirements) of the
brain and must be treated promptly.
Fluid and electrolyte abnormalities

39
Hypotonic fluids must be avoided in children with head injury as a fall in plasma sodium
will exacerbate cerebral oedema. Plasma sodium and plasma osmolality must be
maintained within the high normal range (aim for plasma sodium 150 mmol/l in severe
head injury).
Children with traumatic brain injury are susceptible to a variety of abnormalities of
plasma sodium:
Inappropriate antidiuretic hormone (ADH) – hyponatraemia, low plasma osmolality, high
urinary osmolality, normo/hypervolaemia, potential cerebral oedema. Treat with fluid
restriction if asymptomatic or hypertonic saline if symptomatic (1-2 ml/Kg 3% saline
bolus)
Cerebral salt wasting – hyponatraemia, high urinary osmolality, hypovolaemia and
hypotension. Treat with normal saline bolus
Diabetes insipidus – hypernatraemia, high plasma osmolality, low urinary osmolality,
hypovolaemia and hypotension
Diabetes insipidus occurs as a result of failure of blood supply to the posterior pituitary
with loss of ADH production in the posterior pituitary – it should be treated by
administration of DDAVP (Desmopressin), however, DI is a late sign in head injury and
often heralds brain stem death.
Coagulopathy
Penetrating injury is associated with release of brain tissue into the circulation, which
may result in disseminated intravascular coagulation.
Autonomic changes
Intense peripheral vasoconstriction and hypertension are often seen in severe head injury.
Neurogenic pulmonary oedema
Severe head injury may be associated with sudden onset of severe pulmonary oedema
leading to hypoxia.
Analgesia and anaesthetic agents
Pain and anxiety increase metabolic demands and should be treated, avoiding excessive
doses of agents that may cause respiratory depression, or alternatively, ventilating the
patient electively to allow for this.
A small percentage of children with head injury may require surgery to evacuate
intracranial haematoma. Volatile anaesthetic agents reduce the metabolic requirements
but increase cerebral blood flow and ICP. Halothane increases ICP more than isoflurane
and should be avoided if possible.
Intravenous anaesthetic agents reduce the cerebral metabolic rate, and also reduce
cerebral blood flow and ICP, with the exception of ketamine, which increases ICP.
Ketamine should be avoided in head injury patients if possible; thiopentone or propofol
are the intravenous agents of choice, along with judicious doses of opioids to obtund the
reflex cardiovascular responses to intubation.
Suxamethonium will increase ICP transiently only. Suxamethonium is indicated for rapid
sequence induction in head injured patients.
ASSESSMENT OF NEUROLOGICAL DISABILITY
All children suspected of head injury should have regular neurological assessments and
assessment of pupil responses.
Modified Glasgow Coma Scale for Children

40
Rapid neurological assessment of patients with head injury may be made using the
AVPU score (Alert, responds to Verbal commands, responds to Pain, Unresponsive). The
Glasgow coma scale is a more sophisticated method of assessing and describing level of
consciousness and has been modified for use in children (table 2). The motor response is
the most accurate predictor of poor outcome; any child with a reduction in motor score
below “localizing pain” should be treated as having a severe head injury.

Table 2. Modified Glasgow coma score for children


Score Infant/nonverbal child Verbal child
Eye Opening: 4 Spontaneous Spontaneous

3 To speech To speech
2 To pain To pain
1 None None
Verbal: 5 Babbles and coos normally Oriented

4 Spontaneous irritable cries Confused


3 Cries to pain Inappropriate words
2 Moans to pain Incomprehensible
1 No response sounds
No response
Best motor 6 Normal spontaneous Obeys command
response: movement
5 Localizes pain
4 Withdraws to touch Flexion withdrawal
3 Withdraws to pain Abnormal flexion
2 Abnormal flexion to pain Extension to pain
1 Extension to pain No response
No response
Table 3. Severity of head injury
Severity of head injury Glasgow coma score
Mild 13-15
Moderate 9 -12
Severe </=8
Pupil responses
Pupil responses are an important clinical sign and should be assessed regularly in all
children with head injuries.
· Normal response – pupils react symmetrically to light and accommodation
· Bilateral fixed and dilated pupils – inadequate cerebral perfusion, possibly irreversible
brain injury
· Bilateral pupil constriction – early brain herniation through the foramen magnum, injury
to the brainstem, side effect of opioids
· Unilateral fixed and dilated pupil – herniation of the brain through the tentorium
cerebelli within the skull on the same side, optic nerve injury
· Unilateral pupil constriction – unilateral brainstem injury, Horner’s syndrome

41
MANAGEMENT
Children with head injury must be assessed and appropriate management started
immediately. Delays, particularly in management of hypoxia and hypotension, worsen
outcome.
In a child thought to have suffered a minor head injury only, the following signs are
worrying and merit admission of the child to hospital for close monitoring, including
regular neurological assessments:
· Patients who are not fully alert
· Persistent vomiting, severe headaches
Children with moderate to severe head injury should all be admitted for rapid assessment.
The following features in the history and clinical condition indicate the possibility of a
severe head injury:
· History of a fall from a height, a high-speed road traffic accident, or road traffic
accident where the child is a pedestrian or a cyclist
· Loss or reduced level of consciousness
· External sign of skull fracture, including base of skull fracture (‘panda eyes’, blood or
CSF in the ear, bruising of the mastoid process behind the ear)
· A Glasgow coma score of </= 8 or loss of ability to localise pain on the motor category
Immediate management of a child with moderate to severe traumatic brain injury
Assess the conscious level and pupil responses at the same time as attending to ‘ABC’ as
follows:
· Airway with cervical spine immobilisation
· Breathing and ventilatory control
· Circulation and control of obvious external bleeding
· Disability and neurological status, including pupil responses
· Exposure – secondary survey with top to toe examination to detect associate injuries
(consider non-accidental injury)
The first priority is stabilisation of the airway and the cervical spine.
Airway patency should be established, taking care of the cervical spine (jaw thrust +/-
airway adjuncts), and high flow oxygen should be administered. Cervical spine injuries
are uncommon and only occur in about 1% of severe head trauma patients. However,
children should have cervical spine immobilisation if there is a suspicion of cervical spine
injury, for instance a significant mechanism of injury, a history of loss of consciousness,
neck pain or tenderness, another severe (“distracting”) injury or a history of intoxication.
A child who is restless and disoriented should not be forced to wear a rigid cervical collar
or forcibly restrained as this may increase fighting, anxiety and intracranial pressure.
Breathing
Should be assessed by clinical examination of respiratory pattern and rate, chest
auscultation and oxygen saturation. A Chest X-ray should be obtained as part of the
initial essential imaging. Arterial blood gas analysis should be obtained if possible.
The child will require intubation and ventilation in the ICU in the following situations:
· Reduced level of consciousness with a Glasgow coma score ?8
· Inadequate breathing (saturation persistently <95% in oxygen), hyperventilation or
hypoventilation (PaO2 <9kPa in air/<13kPa in oxygen or PaCO2 <3.5/>6kPa)
· Loss of protective laryngeal reflexes
· Significant facial or neck injuries

42
· Seizures
An intubated child with a head injury in the ICU will require sedation and analgesia;
midazolam and morphine are the usual drugs of choice. A muscle relaxant may be
required if it is difficult to control the ventilation. The child should be ventilated to obtain
a normal PaCO2. An appropriately trained nurse or anaesthetist should always be present
with the child.
Circulation
Hypotension must be actively sought and treated. Venous access should be established
early and blood samples taken for full blood count, electrolytes and group and save. Two
cannulae should be inserted, and intraosseous access or venous cutdown should be
considered if venous access is difficult.
A useful formula for expected blood pressure in children is:
Expected systolic BP (mmHg) = 80 + (age in years x 2).
As discussed above, a higher than normal blood pressure should be maintained to ensure
adequate cerebral perfusion.
Normal saline or Ringers are the fluid of choice for initial resuscitation. An initial fluid
bolus of 10 ml/kg should be given, followed by another 10 ml/kg if the child remains
hypotensive. Overhydration must be avoided, as this will promote cerebral oedema
formation. If the haemoglobin is less than 7g/dl then extracranial causes of blood loss
should be considered (or chronic anaemia). The child may require blood transfusion. Of
note however, an intracranial bleed in a neonate may cause hypovolaemia and a fall in
haemoglobin (before signs of raised ICP become apparent).
Disability and exposure
Assess the GCS and pupil responses. Other injuries should be sought and treated. Scalp
lacerations may result in significant blood loss. If the child remains cardiovascularly
unstable and requiring volume resuscitation, consider other sites of blood loss: chest,
abdomen, pelvis or major limb fracture.
Indications for CT scanning
A CT scan is the gold standard imaging in assessing patients with head injury, if
available. The CT scan will pinpoint a surgically remedial lesion such as subdural or
extradural haematoma, and give an indication of severity of brain injury (presence of
cerebral oedema, contusions, intracerebral bleeds etc). However, this is an expensive
facility and may not always be available at the receiving hospital. The indications for CT
scanning are as follows:
· A clinical suspicion of severe head injury, such as reduced conscious level, amnesia,
abnormal drowsiness
· Skull fracture, either basal or open or depressed skull fracture
· Seizures, other neurological deficits or persistent vomiting
· A dangerous mechanism of injury
· Suspicion of non-accidental injury
As cervical spine X-rays are rarely adequate in children wearing cervical collars, a CT of
the head should be extended to include the neck too.

Indications for referral for surgery


10 – 20% of patients with severe traumatic brain injury have a surgically treatable
condition such as extradural haematoma requiring burr hole; neurosurgical intervention

43
may be life saving in this situation. A surgical opinion should be sought if the CT scan
shows an intracranial haematoma or there is clinical suspicion of an intracranial
haematoma, such as progressive focal neurological signs. A surgical opinion should also
be sought in the case of depressed skull fracture, CSF leak or penetrating injury. Tertiary
neurosurgical centres are able to offer interventions to manage severe raised intracranial
pressure (see below).
Intensive care management
The primary goal of intensive care management in severe traumatic brain injury is
prevention of secondary brain injury by maintaining adequate cerebral perfusion and
oxygenation and controlling raised intracranial pressure. Children with severe traumatic
brain injury should be transferred to an intensive care unit if at all possible. Secondary
and tertiary centres will be able to undertake advanced techniques such as invasive blood
pressure monitoring, use of inotropes and intracranial pressure monitoring, as well as
CSF drainage or decompressive craniotomy for patients who deteriorate due to diffuse
cerebral oedema. Such treatments are expensive and will not be possible in most resource
poor environments. General supportive measures should be possible in all intensive care
units.
General intensive care management
All patients with head injury should have simple measures instituted as follows:
· Nurse the child in 30° head up position with neutral head positioning
· Maintain SpO2 >95%
· Maintain CO2 4.5-5.0 kPa
· Maintain blood pressure in the high/normal range
· Avoid excessive fluid loads
· Avoid hypotonic fluids containing dextrose. Aim for plasma sodium 145-150 mmol/l
· Use inotropes if necessary to maintain blood pressure (noradrenaline)
· Provide adequate sedation and analgesia
· Maintain blood sugar in normal range
· Maintain normal temperature, treat pyrexia aggressively
· Control seizures
· Provide adequate nutrition via a nasogastric tube (start early).
Special treatment for raised intracranial pressure, tertiary centres:
· Institute intracranial pressure monitoring using a bolt or extradural or intraventricular
catheter if possible. Rising intracranial pressure should be managed aggressively,
particularly ICP>20mmHg
· CSF drainage via intraventricular drain
· Mild hyperventilation to PaCO2 < 4.5 kPa
· Consider osmotic therapies to reduce intracranial pressure such as mannitol or 3%
hypertonic saline
· Consider barbiturate-induced coma with refractory intracranial pressure elevation if the
child is cardiovascularly stable.
· Profound hyperventilation to PaCO2 < 3.9 kPa may be used temporarily pre-craniotomy
· Decompressive craniectomy may be considered in children with diffuse cerebral
oedema, within 48 hours of injury, with no sustained episodes of intracranial pressure
>40mmHg and secondary clinical deterioration with evolving cerebral herniation
Conclusion

44
Immediate airway management (with cervical spine control) and cardiopulmonary
resuscitation are the most important early steps in management of traumatic brain injury
in children. The importance of avoiding hypotension and hypoxia and of detection and
rapid treatment of surgically remediable lesions cannot be over emphasised. Children are
best managed in experienced paediatric trauma centres if at all possible. This may require
transfer to another centre over long distances. Excellent communication and
multidisciplinary approach is essential to produce the best outcomes.
References and further reading:
1. Hatch and Sumner. Trauma and transport Textbook of Paediatric Anaesthesia (3rd
Edition): 635-645,
2. Hatch and Sumner. The central nervous system Textbook of Paediatric Anaesthesia
(3rd Edition): 95-108,
3. Orliaguet, Meyer and Baugnon. Management of critically ill children with traumatic
brain injury, Paediatric Anaesthesia 2008;(18):455-461
4. Giza, Mink and Madikians. Pediatric traumatic brain injury: not just little adults.
Current Opinions in Critical Care 2007;(13):143-152
5. Lam and Mackersie. Paediatric head injury: incidence, aetiology and management.
Paediatric Anaesthesia 1999; (9): 377-385

2. SPINAL CORD COMPRESSION DUE TO TUMOURS.

HISTORICAL PERSPECTIVE

The first successful operation for a spinal cord tumour was performed in 1888 by Victor
Horsley and Gowers at the National Hospital for Neurology and Neurosurgery, Queen’s
Square, London. It was an intradural extramedallary tumour. Surgery was based entirely
on clinical diagnosis and localization. In 1907, Dr. Elsberg performed the first successful
operation for an intra medullary tumour. Further advances were to await the introduction
of myelography by Walter Dandy in 1919. This allowed pre-operative localization of the
tumour to be done. In 1921, Sicard and Forestier introduced positive contrast
myelography as opposed to air-myelography which had been introduced by Dandy. After
this it was possible to accurately localize spinal cord lesions before surgery. Other
advances of importance were the introduction of bi-polar coagulation in the 1940’s
(which was later modified by Malis) and the introduction of the operating microscope in
1964.

INCIDENCE

Spinal cord tumours are rare. Figures available in the Western literature indicate that
there are three to ten spinal cord tumours per hundred thousand population. They make
up about 1/5 all the tumours of the Central Nervous System. The commonest spinal cord
tumours are neurilemomas (30%), meningiomas (25%), ependymomas (13%), and
astrocytomas (7%). Other rarer tumours which may cause spinal cord compression are
sarcomas (12%) vascular tumours i.e. hemangioblastomas & a-v malformation (6%) and

45
other rarer tumours such as dermoid, epidermoid, teratomas and chordomas (8%). 16%
of spinal cord tumours are intramedullary and 84% are extramedullary. In the Western
World, metastatic tumours are the commonest cause of spinal cord compression.
Mwang’ombe & Ouma (EAMJ July 2000) showed that spinal cord tumours accounted for
about 15% of all CNS tumours treated at the Kenyatta National Hospital between 1985-
1994. Most of the patients in their study had total paralysis of the limbs at the time of
presentation. Meningiomas and neurofibromas were the commonest cause of cord
compression. The mean grade of presentation was 37 years.

SURGICAL ANATOMY

The spinal cord is an ovoid neural structure within the vertebral canal which extends from
the foramen magnum to the inter-vertebral disc between the first and second lumbar
vertebra. (L 3 at birth). The cord is enlarged in two areas, cervical enlargement and
lumbar enlargement. There are 31 pairs of spinal nerves; 8 cervical, 12 thoracic, 5
lumbar, 5 sacral and 1 cocygeal. The first pair exits between the atlas and the skull. All
the 31 pairs of spinal nerve have dorsal root ganglia except C1 and coccygeal nerve.
Spinal cord segment C1 and cervical vertebra C1 are at the same level. After this the
cord level becomes progressively higher that the corresponding vertebral level. The cord
is surrounded by fibrous tissue called pia-matter. It is suspended within the vertebral
canal by 21 pairs of lateral dentate ligaments which are attached to the dura matter. The
dentate ligaments lie midway between the ventral and the dorsal roots. The filum
terminale runs from the posterior inferior aspects of cornus medullaris to inferior
insertion on the dorsal aspect of the coccyx.

BLOOD SUPPLY

There are 31 pairs of radicular artery branches which enter the vertebral canal through
inter-vertebral foramen. These are of three types; the proper radicular branches which
end within the roots, the pia matter radicular branches and the spinal branches which
enter the cord. The blood supply of the cord may be divided into three large arterial
areas.
1. Cervical thoracic which lies between cervical and T2/3 junction; this is supplied by
the anterior spinal artery from the vertebral artery.
2. The mid thoracic region between T3 and T8. This is supplied by a single radicular
artery from T7.
3. Thoraco-lumbar region between T8 and lumbar enlargement. This is supplied by a
single radicular artery from T12/L1 junction.

PATHO-PHYSIOLOGY & CLINICAL PRESENTATION

A patient with a spinal cord tumour may present at any of these stages;

1. First Stage.
In the early stages the patient presents with root and segmental sensory loss or motor
disturbance.

46
2. Second Stage.
Later, the patient presents with features of Brown-Sequard syndrome or incomplete
transection syndrome.

3. In the late stages patients present with complete transection.


Clinical examination will reveal the following features:
a) Disturbances of motor function. This may be gradual in which case the patient
presents with spastic paresis or rapid in which case the patient presents with flaccid
paresis. Gradual onset is common in slow growing tumours i.e. meningiomas and
neurilimomas. Rapid presentation is common in first growing tumours e.g. metastatic
tumours. Patient will usually complain of progressive weakness of the extremities
and tiredness.
(b) Disturbance of sensory long tracts (collectively or individual). This may involve the
posterior column (vibration and positions sense are effected) or the antero-lateral
tracts (loss of pain and temperature). Compression of the dorsal surface of the cord
by meningiomas and neurilimomas will result in affection of all modalities of
superficial sensation, position and vibration sense. This is common in extra
medullary tumours. In intramedullary tumours the patient may present with
paresthesias and numbness which may be associated with painful dysethesia.
Patients with intra-medullary tumours may also present with bilateral dissociative
Sensory loss; loss of pain and temperature but touch and position intact.
c) Root symptoms. This may be unilateral pain at the level of the dermatome supplied
by the root. The pain is worse at night and when the patient is supine or during
valsalva manouvre. Other features which may be observed are fasciculations,
paresis and amyotrophy.
Clinical presentation may be further categorized at the cord level into;
1. High cervical cord. In tumours in this region patient may present with neck pain,
headache in greater occipital nerve distribution, respiratory distress/weakness and
intercostals breathing which is due to the involvement of the phrenic nerve.
2. Lower cervical tumours. Patient presents with pain in the arm or shoulder. This
may resemble cervical disc disease and cervical spondylosis with myelopathy.
3. Thoracic cord tumours. Patients present with funicular pain (dysesthesia), long tract
motor disturbances, root symptoms, Beevor’s sign may be positive (presence of
T9 & T10 roots, absence of T11 & T12 leads to upward movement of umbilicus with
Contraction of abdominal muscles). A complete sensory deficit usual indicates the
Location of the tumour.
4. Conus medullaris/Cauda equina tumours. In tumours of this region there is early loss
of bladder and bowel control. There is also symmetrical saddle anaesthesia
Commonly seen in ependymomas.

INVESTIGATIONS

1. Plain radiography. The following features may suggest the presence of a


spinal cord tumour; widening of the interpeduncular distance, widening of the neural
foramina, scalloping of the vertebral bodies, loss of a pedicle, or evidence of bone

47
destruction or blastic changes in the vertebral bodies (malignant extraduaral lesion).
2. Myelography. Lumbar puncture should not be done before myelography as it may
cause cord shift and incarceration and collapse of subarachnoid space. Do
Queckenstedt test, if there is free flow remove csf for analysis, if there is a block do
not remove (Froin’s syndrome, high protein and low cell count; suggestive of block
due to tumour). Features which may be observed during myelography and which are
suggestive of spinal cord tumour are;
Extradural tumours show features of paint brush appearance or hour glass deformity.
Intradural tumours show cupping defect.
Intramedullary tumours shows fusiform widening of the cord shadow
3. Computerized tomography (CT) scan/myelography. This is the investigation of
choice where available.
4. Magnetic resonance imaging (MRI). This is superior to CT scan for the study of
spinal cord pathology, but is rather expensive and rarely available in developing
countries.
5. Spinal angiography. This is important where one suspects the presence of
haemangioblastomas, arteriovenous malformations and occipital meningiomas.

TREATMENT
SURGERY

The definitive treatment for spinal cord tumours is surgical excision through
laminectomy. Total removal is possible for extradural and intraduaral extamedullary
tumours. Total removal also may be possible in some intramedullary tumours.

EPENDYMOMA.

Most frequent intra-medullary spinal cord tumour in adult patients in Western World.
MRI does not always differentiate between astrocytoma and ependymoma. Therefore
surgery is always necessary so as to rule out epenymoma. Post-operation radiotherapy
not necessary. Symptoms are usually subjective peripheral sensory disorders of the limb
or trunk. (MRI has a high degree of accuracy for visualizing non-glial tumours e.g.
haemangioblastomas, dermod and epidermod tumours and lipomas but not epenymomas).
Classified as Grade II (WHO) tumours. They are benign slowly growing tumours that
may grow to considerate size e.g. affecting entire spinal cord (holocord ependymoma),
before becoming clinically detectable. Most commonly affect cervical cord, with
extension to thoracic area or medulla, followed by thoracic and then conus medullaris.
Surgical Technique:
Somato-sensory potentials are recorded during surgery.
Intra-operative ultrasonography.
Microscopic dissection through dorsal median sulcus (post-spinal vein).
Spread posterior columns apart and retract them.
Biopsy sample is obtained and histological examination.
If inftrating or malignant tumour; further surgery.
Tumour removal.

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ASTROCYTOMAS

These are less common than ependymomas. They may present as microcysts or large
syrinxes. Treatment is decompression with aspiration of cysts followed by radiotherapy.

NEURILEMOMAS/NEUROFIBROMA

These are the most common tumours making up 30% of all spinal cord tumours. Male to
female ratio is equal. 70% are intradural extramedullary and 15% are extradural. 14%
are dumbbell and 1% are intramedullary. Thoracic site is the commonest followed by
cervical, but rare in lumbar region. They are found mainly in the 4th & 5th decade.

MENINGIOMAS

These are the second most common tumours making up approximately 20% of all spinal
cord tumours. 80% are found in women and occur mainly in the 4th, 5th and 6th decade.
Two thirds occur in the thoracic area. 85% are intradural/extramedullary. 15% are
extradural.
METASTATIC TUMOURS

These are most common in patients over 50 years. Mainly from lung, breast, prostate,
kidney, sarcomas and lymphomas. If the primary tumour is known and there is no motor
weakness the patient is put on steroids and radiotherapy. In cases of prolonged
paraplagia surgery is useless. Surgery is also of no benefit in patients in poor general
condition with wide spread metastases.
Other causes of spinal cord compression of surgical importance are infection and trauma.

TUMOURS OF THE SPINAL CORD IN CHILDREN

Incidence: Same as in adults: One sixth are affected male that of intracranial tumours
more commonly than females by ratio of approximately 7:5.
Patients with Von Recklinghawsens disease have an increased incidence of gliomas,
neurinomas, and meningiomas.

Commonly present between the ages of 3 and 5 years because of the high incidence of
congenital neoplasms in childhood.

Distribution of intraspinal tumours in children differs from that in adults and there is a
greater number of intrinsic spinal cord neoplasms (intramedullary) especially
astrocytomas (35 percent) and lesser incidence of intradural-extramedullary neoplasms.
Such as neurinomas and meningiomas (30 percent) while extradural tumours make up 30
percent. (In adults intradural extramedullary, 60 percent, extradural 25 percent and
intramedullary 15 percent).

40 Percent of intraspinal tumours in children are found in the thoracic area and 60 percent
evenly distributed in the cervical and lumbosacral regions.

49
70 percent of intraspinal tumours in children are benign and slowly growing. The most
common histological types are astrocytomas (25 percent), dermoids (15 percent),
epidermoids (10 percent) and lipomas (5 percent).

Secondary medulloblastomas that seed the spinal subarachnoid space are seen.

Symptoms and signs.


The symptoms and signs produced by these tumours may be observed in conditions such
as cerebral palsy, congenital torticollis, idiopathic scoliosis, spina bifida occulta,
transverse myelitis, Guillain Barre Syndrome, brain tumour, brachial plexus palsy,
idiopathic enuresis, meningitis and appendicitis.

Most common manifestation is motor weakness, (two thirds of the cases). This may
present as increased fatigability while running or climbing stairs.
Back or root pain may be seen in 40 to 50 percent of children. In older patients, it is
accentuated by coughing, sneezing, flexion of the spine, or strength leg raising.
Sensory disturbances and bladder and bowel dysfunction may be seen in 30 percent of
children at the time of clinical presentation. Thorough sensory examination may be
difficult in the very young subjects, but perianal anesthesia and segmental hypalgesia
should be looked for. Delayed development of sphincter control or loss of established
bladder or bowel control may be significant in the history.
Tumours in the cervical and thoracic segments of the spinal cord produce a small spastic
bladder. Patient voids in frequent small amounts. Tumours in the conus medullaris and
cauda equina produce a large flaccid bladder with stress incontinence of urine and faeces.

Musculoskeletal deformities as a result of neuromuscular imbalance and somatic growth


are seen in slowly growing neoplasms. These may be in the form of torticollis, scoliosis,
kyphosis and foot deformities. Hydrocephalus may be seen in high cervical
intramedullary gliomas.

Recurrent meningitis may be seen where there is a connecting sinus tract. The organisms
are usually Escharichia Coli and Staphylococcus aureus.
3. DIAGNOSIS AND MANAGEMENT OF BRAIN TUMOUR
CLASSIFICATION OF BRAIN TUMORS
TUMORS OF NEUROGLIAL CELLS
A. Tumors of Glial Cells
Astrocytic tumors
Astrocytoma
Glioblastoma multiforme
Oligodendroglioma
Ependymoma - choroid plexus papilloma
B. Neuronal Tumors
Ganglioglioma
Gangliocytoma
Central neurocytoma

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C. Embryonal Tumors
Medulloblastoma
MENINGEAL TUMORS
Meningioma
TUMORS OF CRANIAL AND SPINAL NERVES
Schwannoma
Neurofibroma
MESENCHYMAL TUMORS
Sarcoma
Hemangioblastoma
CEREBRAL LYMPHOMAS
GERM CELL TUMORS
Teratoma
Craniopharyngioma
TUMORS OF THE PITUITARY GLAND
Pituitary adenoma
METASTATIC TUMORS
WHO GRADING SYSTEM
Grade I-Pilocytic
Benign cytological features-see below
astrocytoma
Grade II-Low-grade Moderate cellularity-no anaplasia or mitotic
astrocytoma activity
Grade III- Anaplastic
Cellularity, anaplasia, mitoses
astrocytoma
Same as Grade III plus microvascular
Grade IV-Glioblastoma
proliferation and necrosis

Neuroepithelial tumors
Astrocytoma
Astrocytomas, which arise from astrocytes, are the most common primary brain tumor.
Astrocytomas are histologically graded based on cellularity, anaplasia, mitotic figures,
endothelial proliferation and necrosis. Well-differentiated astrocytomas (WDA) have
mild hypercellularity and minimal nuclear pleomorphism. They typically occur in
children and young adults who present with a seizure or headaches. On CT or MRI scan
they usually appear as a non-enhancing mass lesion. Anaplastic Astrocytomas (AA) have
moderate cellularity and nuclear pleomorphism with mitotic activity. Moderate
endothelial proliferation can be present but no necrosis. These lesions are typically found
in mid-life as enhancing lesions with mass effect. They often present with seizures,
headaches and/or focal neurological findings. Glioblastoma multiforme (GBM) is
characterized by hypercellularity, dramatic nuclear pleomorphism, endothelial
proliferation, mitotic figures and necrosis. These patients are usually older adults who
present with headaches, seizures or focal neurologic findings. On CT or MRI scan a
GBM typically appears as an irregular ring-enhancing lesion with significant mass effect
and edema.

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Survival correlates with grade: WDA 5-10 years, AA 2-3 years and GBM 1-1.5 year.
Astrocytomas can initially present as a low-grade tumor and subsequently convert to a
higher grade. De novo AA and GBM tumors occur as well.
Treatment is based on grade. Controversy exists for the optimal treatment of WDA. Most
agree that a WDA in non-eloquent brain with mass effect should be resected. A
stereotactic brain biopsy for diagnosis is an option for symptomatic lesions in areas of the
brain where open surgery carries an increased risk of causing a deficit. Radiation is
controversial in stable tumors but is often used if growth is demonstrated. AA and GBM
are typically treated by surgical resection followed by radiation and BCNU
chemotherapy. Surgery is indicated for diagnosis, to relieve mass effect, and, possibly, to
decrease the "tumor burden". AA or GBM in the motor strip, language areas or other
eloquent brain regions are often biopsied rather than resected because of the high risk of
surgery in these areas. It has been difficult to prove that the extent of tumor resection has
an effect on patient survival. As a rule, surgery is never curative. BCNU impregnated
wafers implanted in the tumor bed after resection of a recurrent AA or GBM have proven
to be efficacious. Recurrence within a centimeter or two of the resection site is typical
regardless of the treatment given after surgical resection. Surgery for recurrent AA and
GBM is controversial. Most surgeons agree that reoperation is indicated for the relief of
headaches or neurological deficits due to mass effect.
Pathologically, several types of astrocytomas exist, such as fibrillary, protoplasmic and
gemistocytic astrocytomas. Glioblastomas also have giant cell and gliosarcoma variants.
A separate group of astrocytoma is the juvenile pilocytic astrocytoma (JPA). This group
is either not graded or considered Grade 0. JPA are distinct because they behave in a
more benign fashion and when completely resected can be cured by surgery alone. The
are typically discrete cystic lesions with an enhancing mural nodule. Histologically, JPA
are composed of loose and dense regions of stellate astrocytes. These have Rosenthal
fibers that indicate slow growth. JPA are typically found in children and young adults.
They tend to occur in the cerebellar hemisphere, optic nerve, hypothalamus and
brainstem. Cerebellar JPA often present with signs of increased intracranial pressure
(headache, nausea, vomiting) due to hydrocephalus. JPA also can present with cerebellar
dysfunction such as gait ataxia or ipsilateral extremity dysmetria. Rarely, JPA can
undergo malignant degeneration. Subarachnoid seeding does occur rarely with JPA and
probably carries a poorer prognosis. This has been seen with hypothalamic tumors. Optic
nerve JPA and WDA tumors are associated with neurofibromatosis type II.
Pleomorphic xanthoastrocytomas are astrocytic neoplasms found in young adults with a
long history of seizures. They are usually superficial in the cerebral cortex and may
consist of a mural nodule associated with a cyst. They are typically slow growing but
malignant transformation does occur. Subependymal giant cell astrocytomas are typically
found at the foramen of Monro in patients with tuberous sclerosis.
Gliomatosis cerebri is a condition where there is diffuse infiltration of the entire brain
with an astrocytic tumor.
Oligodendroglial tumors
The majority of oligodendrogliomas present in young adulthood with the onset of
seizures. Radiographically, calcifications are typical. The classic histologic appearance is
of homogeneously appearing cells with a "fried egg" appearance and "chicken wire"
vessel pattern. Similar to WDA, patients can have long term survival. Malignant

52
transformation does occur. These tumors are called anaplastic oligodendrogliomas. In
general, surgical resection is recommended when possible for diagnosis, to relieve mass
effect, and resect as much tumor as safely as possible. Radiation therapy is controversial,
but is probably beneficial. PCV (procarbazine, CNU, & vincristine) chemotherapy has
been shown to be beneficial in the treatment of oligodendrogliomas.
Ependymal tumors
Ependymomas typically arise from the lining of the ventricular system and usually occur
in children and young adults. The floor of the fourth ventricle is a common location.
Ependymomas typically present with hydrocephalus and increased ICP. Presenting
symptoms include nausea, vomiting, headache, gait ataxia, diplopia and vertigo.
Ependymomas have a significant potential for CSF seeding and thus "drop metastasis".
Complete surgical resection has been shown to improve survival and should be attempted
if there is minimal brainstem invasion. Postoperative radiation and chemotherapy is
usually administered. No clear consensus exists for grading ependymomas but the term
anaplastic ependymoma is sometimes used for more malignant appearing tumors.
Paradoxically, intramedullary spinal cord ependymomas, which are histologically
identical, may be cured by surgery alone.
Myxopapillary ependymomas are a variant of ependymomas. This tumor occurs in the
conus or filum terminale of the spinal cord. Complete resection is probably curative.
Subependymomas occur in anterior lateral ventricles or posterior fourth ventricle. They
are benign slow growing tumors that are typically found incidentally at autopsy. However
subependymomas can cause hydrocephalus from obstruction of cerebrospinal fluid
pathways. Symptomatic or enlarging tumors should be removed. In elderly patients
insertion of a VP shunt is a viable option if obstruction of CSF pathways is present.
Mixed gliomas
Mixed gliomas occur and appear histologically as a combination of neoplastic
oligodendrocytes and astrocytes. These tumors are referred to as oligo-astrocytomas or
anaplastic oligo-astrocytomas. Often the name is shortened to the dominant cell type
only.
Choroid plexus tumors
Tumors of the choroid plexus are called choroid plexus papillomas (CPP). In children
less than 2 years of age, they usually are located in the lateral ventricle and present with
hydrocephalus. In adults CPP usually occur in the fourth ventricle, foramen of Luschka
or cerebellopontine angle. The treatment is surgical resection, though elderly patients
with an asymptomatic cerebellopontine angle tumor may be followed with serial imaging
studies. Recurrence should be treated aggressively with reoperation, when possible, due
to a favorable prognosis. One or two percent of choroid plexus tumors are carcinomatous.
Choroid plexus carcinoma carries a poor prognosis.
Neuronal and mixed Neuronal-glial tumors
Gangliocytoma is a tumor composed of large abnormal mature neurons. They are
primarily supratentorial with the most common location being the temporal lobe. The
majority of patients are in their first two decades of life. Surgical resection, when
complete, is curative. Variable radiographic features occur, ranging from an enhancing
mural nodule to a ring enhancing mass with calcifications.
Dysplastic gangliocytoma of the cerebellum (Lhermite Duclos Disease) is a non-
neoplastic mass of hypertrophic granular cell neurons which expands the cerebellar folia.

53
These tumors can cause mass effect and hydrocephalus and typically occur in young
adults. Resection (total or subtotal) and/or shunting are therapeutic options.
Dysembryoplastic neuroepithelial tumors (DNET) are a "hamartomatous", supratentorial,
predominantly temporal lobe lesion composed primarily of glial cells. They usually
present with seizures. Radiographically these tumors often lack edema and have a
multinodular appearance. Inner table skull erosion or deformation may be present.
Gangliogliomas are tumors consisting of large mature neurons and a neoplastic glial
component. This tumor affects patients of all ages with the majority diagnosed in young
adults who often have a long history of seizures. Surgical resection even if subtotal can
be curative. Surgery is recommended for diagnosis and, on occasion, for control of
seizures.
Pineal tumors
Pineal tumors are tumors arising from pineocytes. The well-differentiated pineocytoma
occurs in mid-life as a discrete contrast enhancing mass in the posterior third
ventricle/pineal region. The poorly differentiated pineoblastoma has a similar location
and enhances with contrast but shows signs of local invasion and is prone to CSF
dissemination. The complex of pineoblastoma and bilateral retinoblastoma is called
trilateral tumor. In general, pineocytomas have a good prognosis while pineoblastomas
with subarachnoid spread are aggressive and carry a poor prognosis. Pineal tumors often
present with hydrocephalus due to obstruction of the aqueduct of Sylvius. Compression
of the dorsal midbrain by a pineal tumor can result in Parinaud’s syndrome of pupillary
mydriasis, paralysis of upgaze, and convergence retractorius.
Embryonal tumors
Neuroblastoma is a small cell neoplasm with neuroblastic differentiation arising in the
deep cerebral hemispheres of young children (<5 yrs. of age). A variant is the
ganglioneuroblastoma that has a preponderance of ganglion appearing cells.
The olfactory neuroblastoma (esthesioneuroblastoma) is a neuroblastic tumor arising
from the nasal epithelium with cribiform plate involvement. There is a bimodal age
distribution in adolescents and older adults. Patients present with nasal obstruction and or
epistaxis. Complete surgical resection with combined cranio-facial resection is the
treatment of choice, with a generally favorable prognosis. Adjuvant radiation therapy is
generally recommended.
Ependymoblastoma is a rare small cell embryonal neoplasm with prominent
ependymoblastic rosettes. It typically occurs in the cerebrum of children less than five
years of age. Its propensity for craniospinal dissemination often leads to death within a
year.
Retinoblastoma is a retinal neoplasm that occurs in children < 3 years of age.
Ophthalmoscopic exam is diagnostic, giving the characteristic white reflex. These tumors
have both hereditary (earlier onset and bilateral) and sporadic forms. Surgical resection
with or without radiation can be curative.
Medulloblastoma or primitive neuroectodermal tumor (PNET) of the cerebellum is a
small cell neoplasm believed to arise from the external granular layer of the cerebellum.
This tumor arises in the vermis of children and young adults although cases in older
patients have been reported. Radiographically, these are homogeneously enhancing
masses of the cerebellar vermis. CSF tumor seeding can produce drop metastases, even at
the time of diagnosis. Surgical resection, combined with radiation and chemotherapy,

54
may lead to significant long-term survivals. Rarely, metastasis to bone, lymph nodes and
lung have been reported. Variants include desmoplastic medulloblastoma,
medullomyoblastoma, and melanocytic medulloblastoma. Medullomyoblastoma occurs
in children, with a propensity for boys. Cerebral (supratentorial) and spinal PNET’s
occur, but with much less frequency.
Mesenchymal tumours: Haemangioblastoma
Hemangioblastoma is a benign tumor of middle age. In fact, it is the most common
primary intra-axial tumor of the posterior fossa in adults. About 20% are associated with
Hippel-Lindau disease, and hereditary factors have been implicated in another 20%. The
cerebellum and vermis are the common sites, but hemangioblastomas can also be found
in the medulla and spinal cord. Multiplicity is a well-known feature but is present in only
about 10% of cases. Histologic examination reveals a meshwork of capillaries and small
vessels.
The classic MR appearance of hemangioblastoma is a cystic mass with a brightly
enhancing nodule. About 60% are cystic, so solid lesions are not uncommon.
Calcification is rare. Hemangioblastomas are sharply marginated and induce minimal
surrounding parenchymal reaction. The tumor nodules are hypervascular and the vascular
pedicle often produces a characteristic flow void on MR.
Von Hippel-Lindau Disease is an autosomal dominant disease, associated with
hemangioblastomas of the cerebellum and retina, cysts of the liver and pancreas,
pheochromocytomas, and tumors of the kidneys. It is linked to VHL, a tumor suppressor
gene on chromosome 3p. The product of this gene is involved in mRNA transcription.
Tumors of cranial and spinal nerves
Schwannomas
Schwannomas (neurinoma, neurilemmoma) are tumors composed of Schwann cells that
arise along cranial or spinal nerves. The vestibular schwannoma (acoustic neuroma) is
probably the most common schwannoma and arises typically from the superior vestibular
nerve. Vestibular schwannomas typically present with tinnitus and sensori-neural hearing
loss. Facial numbness follows when the tumor reaches approximately 2.5 cm. Ipsilateral
coordination difficulties and mild facial nerve weakness typically do not occur until the
tumor diameter is greater than 3 cm. Radiographically, these tumors enhance with
contrast and extend into the internal auditory canal. Complete surgical resection is
curative. Hearing and facial nerve preservation is dependent on tumor size and
preoperative level of nerve function. In older patients or poor surgical candidates,
stereotactic radiosurgery is an effective treatment for tumor size < 2.5cm. Some
proponents of radiosurgery feel that it should be used as the primary treatment for all but
the largest tumors. Bilateral vestibular schwannomas occur with neurofibromatosis type
II.
Neurofibromas
Neurofibromas are a nerve sheath tumor composed of Schwann cells, fibroblasts and
perineural cells. This tumor can occur in isolation or in associated with
neurofibromatosis. This tumor may be solitary, plexiform or occur as a mixed
neurofibroma/schwannoma. These tumors arise from nerves in the subcutaneous tissue or
in the neuroforamena. Classic spinal tumors have a dumbbell appearance when they
extend across the neuroforamena. Surgical resection is indicated in symptomatic lesions.

55
Malignant transformation in neurofibromas is rare but should be suspected with
increasing size or pain.
Malignant peripheral nerve sheath tumors
Malignant peripheral nerve sheath tumors: neurogenic sarcoma, anaplastic neurofibroma,
and malignant schwannoma are rare malignant tumors arising from the non-neural
elements of nerves. These have a poor prognosis with death resulting within the year.
Complete resection is usually not feasible.
Tumors of the meninges
Meningioma (subtypes: meningothelial, transitional, fibrous, psammomatous,
angiomatous, microcystic, secretory, clear cell, choroid, lymphoplasmacyte-rich,
metaplastic variants, atypical, anaplastic).
Meningiomas are typically solitary, benign, slow growing, extra-axial tumors arising
from arachnoid cap cells in the cranium and spine. The most common locations are
parasagittal, convexity, tuberculum sella and sphenoid ridge. A small percentage may be
intraventricular. Meningiomas rarely occur in children but when they do there is a
predilection for the posterior fossa and ventricle. Less than five percent of meningiomas
are malignant, characterized by brain invasion and increased mitotic activity.
Meningiomas are most common in middle age and elderly women. Common symptoms
include headache, seizures, weakness, and mental status changes. Focal neurologic
deficits on presentation depend on the site of origin of the tumor. Radiographically these
tumors are well circumscribed, homogeneously enhancing lesions. There may be
hyperostosis of the underlying skull. There is often a tail of dural enhancement at the
edge of the tumor after contrast administration on imaging studies. The primary treatment
of symptomatic surgically accessible tumors is surgical resection. Surgery if complete is
often curative. Large lesions may require embolization of intra-operatively inaccessible
vascular supply prior to surgical resection to decrease intraoperative bleeding. Small or
asymptomatic meningiomas in older individuals can be followed and treatment
recommended if growth is demonstrated. Radiation therapy and/or radiosurgery are
treatment alternatives for recurrent tumors or if surgery carries an increased risk of
complications. There is an association between meningiomas and neurofibromatosis type
2 and an abnormality in the long arm of chromosome 22.
Cysts and tumor-like lesions
Numerous cysts and tumor-like lesions can occur in the brain including Rathke’s cleft
cyst, epidermoid cyst, dermoid cyst, colloid cyst of the third ventricle, enterogenous cyst
(neuroenteric cyst), neuroglial cyst, other cysts, lipoma, granular cell tumor (choristoma,
pituicytoma), hypothalamic neuronal hamartoma, nasal glial heterotopias.
Tumors of the anterior pituitary
Pituitary adenomas are slow growing, benign tumors that arise in the anterior pituitary
gland. Pituitary tumors are divided into hormone secreting tumors and non-secretors.
Tumors less than 1 cm in diameter are referred to as microadenomas while tumors larger
than 1 cm are called macroadenomas. Prolactinomas are pituitary adenomas that secrete
the hormone prolactin. Prolactinomas often present with amenorrhea in women and loss
of libido in men. The primary treatment for prolactinomas is medication, usually
bromocryptine analogues. Bromocryptine often results in a decrease in tumor size but
does not kill tumor cells. It usually must be continued for life or tumor recurrence is the
rule. Adenomas that secrete growth hormone produce gigantism if present before puberty

56
and acromegaly if present after puberty. ACTH secreting adenomas result in Cushing’s
disease. Hypercortisolism is characterized clinically by centripetal obesity, moon facies,
buffalo hump, glucose intolerance, hypertension and impaired wound healing. Non-
secreting macroadenomas with suprasellar extension can compress the optic chiasm.
Compression of the optic chiasm from below often produces a bitemporal hemianopsia.
Spontaneous hemorrhage or infarction of a pituitary adenoma is referred to as pituitary
apoplexy and can result in sudden visual loss or hypocortisolism. Emergent surgery is
sometimes necessary for pituitary apoplexy.
The primary treatment for patients with secreting pituitary adenomas producing
Cushing’s disease, acromegaly, or symptomatic non-secreting tumors is surgical
resection. Surgery is typically performed through the transsphenoidal route. Cavernous
sinus invasion by adenoma cells often makes a surgical cure difficult. Recurrent tumors
or those with cavernous sinus invasion can be treated with re-operation or radiation
therapy.
Metastasis
Metastases are the most common brain tumor. Tumors that frequently spread to the brain,
in order of decreasing incidence are: lung, breast, skin, colon, and kidney. Commonly,
lung, thyroid, renal cell and melanoma metastases can become hemorrhagic. The
treatment of solitary lesions is surgical resection followed by radiation therapy. For
patients with multiple asymptomatic lesions, surgery is reserved for diagnosis only. For
patients with controlled systemic disease but multiple brain metastases, a large
symptomatic accessible lesion could be considered for resection. Whole brain radiation is
generally given for multiple brain metastases.
4. BRAIN ABSCESS
Brain abscesses arise by several mechanisms including hematogenous spread, penetrating
trauma, surgery, or local spread from the paranasal sinuses, mastoid air cells or emissary
veins. The peak incidence is in young men due to the occurrence of middle ear and
paranasal sinus infections in addition to congenital heart disease. Other predisposing
factors include Osler-Weber-Rendu syndrome with pulmonary arteriovenous fistulae,
endocarditis, congenital heart disease, dental work and immunosuppression. Symptoms
consist of headache, fever, seizures and/or neurological deficit. The majority of brain
abscesses are solitary. Most patients present with signs of mass effect rather than those of
infection. The clinical manifestation is also dependent on factors such as virulence of the
organisms, patient immune status, and the location of the abscess.
Aerobic and anaerobic bacterial abscesses occur. Abscess cultures in one third of patients
grow multiple organisms. Common organisms based on the site of origin include
Streptococcus species from the frontal/ethmoid sinus; Bacteroides fragilis from chronic
mastoiditis/otitis; Staph. aureus or enterobacteriacea following penetrating trauma or
surgery; Strep. viridans and Strep. pneumonia in cases of congenital heart disease; Staph
aureus and Strep. pneumonia in cases of endocarditis. In immunosuppressed patients,
toxoplasma gondii, nocardia, mycobacteria, yeast and fungal abscesses occur. Outside the
US, tuberculomas, cysticercosis, echinococcus, schistosomiasis and strongoloidiasis are
more common.
In the first stage of brain infection, there is inflammation of the brain, termed early
cerebritis. This stage occurs in the first 3-5 days after inoculation. The CT scan
appearance of cerebritis is that of an ill-defined hypodense contrast enhancing area. This

57
coalesces to a late cerebritis stage during days 4-13 with irregular rim enhancement. This
is followed, at approximately day 14, by a collagen reticulum encapsulation with a
necrotic center (early capsule stage) On CT scan or MRI scan this appears as a ring
enhancing mass often with the abscess wall facing the ventricle appearing the thinnest.
The final stage is the late capsule stage in which there is a three-layer capsule: an outer
gliotic layer, a middle collagenous layer and an inner granulation layer. These can persist
for months on imaging studies before ultimate resolution.
Antibiotics are the mainstay of treatment in all cases. Empiric treatment of a presumed
bacterial abscess requires coverage for both aerobes and anaerobes. Surgery is usually
indicated to confirm the diagnosis of an abscess and for culture and sensitivity of specific
organisms. Stereotactic aspiration is the treatment of choice. Aspiration may need to be
repeated before resolution occurs. Often two to three weeks of antibiotic treatment are
needed before a size decrease is seen on imaging studies. In general 4 – 6 weeks of
intravenous antibiotics are often used, followed by a period of oral antibiotics. Patients
with nocardia abscesses, or patients in whom treatment has failed after the third
aspiration, should consider surgical resection when accessible. Often, aspiration alone
can treat significant mass effect and prevent rupture of the abscess into the ventricular
system. Ventricular rupture of a bacterial brain abscess is often fatal.
5. DIAGNOSIS AND MANAGEMENT OF HEADACHES
The major causes of intracranial hemorrhage are vasculopathy in the aged (hypertension
and amyloidosis), aneurysm, vascular malformation, tumor, and coagulopathy.
Hypertension.Spontaneous intracerebral hemorrhage may occur with both acute and
chronic hypertension. Acute hypertension sufficient to produce spontaneous intracerebral
he morrhage is sometimes seen with eclampsia and drug intoxication with
sympathomimetic drugs
Chronic hypertension may lead to vascular changes of the basal perorating arteries
including formation of Charcot-Bouchard aneurysms. The most common locations for
theshemorrhages are (in decreasing order of frequency) basal ganglia, thalamus, pons,
cerebellum, cerebral white matter, brainstem.
Amyloid angiopathy.This is characterized by a deposition of beta amyloid protein within
the meningeal and cortical vessels. The clinical course is characterized by multiple lobar
intracerebral hemorrhages. Risk factors include advanced age and Down’s syndrome.
Definitive diagnosis requires pathologic examination.
Aneurysm. Cerebral aneurysms result from weakening of the wall of major intracranial
arteries. These aneurysms are generally saccular, though fusiform aneurysms also occur.
Saccular aneurysms usually occur at branch points along the arterial tree. When a
saccular aneurysm ruptures, it most commonly produces subarachnoid hemorrhage.
Intracerebral, intraventricular, and subdural hemorrhage are also seen. Common sites of
aneurysm rupture include (in decreasing order of frequency) anterior communicating
artery, posterior communicating artery, middle cerebral artery, basilar artery, vertebral
artery. Multiple aneurysms are seen in approximately one-fourth of patients. Risk factors
include hypertension, smoking, family history, and collagen vascular disease.
Vascular malformation.Vascular malformations include the high flow arteriovenous
malformation, cavernous angioma, venous angioma, and capillary telangiectasias. The
high flow arteriovenous malformation results from a direct communication between the
arterial blood supply and draining veins without normal interposed capillaries. These are

58
usually visible on arteriography. Saccular aneurysm are frequently seen on feeding
vessels. Intraparenchymal hemorrhage is most commonly seen, though subarachnoid and
subdural hemorrhage may occur. Cavernous angiomas consist of irregularly formed
vascular channels, without intervening brain parenchyma. Flow is usually low and these
lesions, therefore, do not routinely manifest themselves on arteriography. Hemorrhage,
when it occurs, is typically intraparenchymal. Family history is a risk factor. Venous
angiomas result from a cluster of normal medullary veins draining into a central enlarged
venous channel. The arterial system is uninvolved. Hemorrhage is infrequent and usually
intraparenchymal. Many are associated with cavernous angiomas. Capillary
telangiectasias are clusters of dilated capillaries with low flow. They are not visible on
arteriography. They are sometimes associated with Osler-Weber-Rendu syndrome, Louis-
Barr syndrome, Myburn-Mason syndrome, and Sturge-Weber syndrome.
Tumor. Both primary and metastatic tumors may hemorrhage. Of the malignant primary
brain tumors, glioblastoma multiforme most commonly presents with hemorrhage. While
lung carcinoma is the most common hemorrhagic cerebral metastasis, melanoma,
choriocarcinoma, and renal cell carcinoma are the most likely metastases to present with
cerebral hemorrhage. Most hemorrhages are intraparenchymal.
Coagulopathy. Even in the absence of underlying cerebral pathology, coagulopathy,
either secondary to underlying medical disease or iatrogenically induced, increases the
risk for intracranial hemorrhage. Particularly in elderly patients, subdural hematoma is
the most common manifestation. Intraparenchymal hemorrhage may also occur,
particularly in the setting of anticoagulation after cerebral infarction.
Subarachnoid hemorrhage. The most common symptom of subarachnoid hemorrhage
is explosive onset of severe headache. The headache is described by the patient as "the
worst headache of my life". The patient may complain of neck pain and stiffness, as well
as photophobia. Low back pain may also be present. Nausea and vomiting may be
present. There may be a history of transient loss of consciousness.
Signs of subarachnoid hemorrhage include nuchal rigidity, positive Kernig’s sign, and/or
positive Brudzinski’s sign. A depressed level of consciousness may also be present.
Ocular manifestations include hemorrhages and papilledema (secondary to elevated
intracranial pressure). Focal neurologic deficit, particularly third nerve palsy ipsilateral to
the site of aneurysm rupture, may be present and in some instances may help to localize
the aneurysm.
Intracerebral hemorrhage. Headache may be present after intracerebral hemorrhage.
Patients with nondominant hemorrhages may complain of weakness or numbness on their
nondominant side. Signs of intracerebral hemorrhage are typically those of hemispheric
neurologic deficit. Patients with dominant-sided lesions are frequently found to have
hemiparesis and hemianesthesia. Dysphasia is usually present, though the patient may
present with frank aphasia. Patients with nondominant lesions typically have contralateral
hemiparesis and hemianesthesia. While speech is frequently normal, neglect may be
profound. If the hemorrhage is large, the patient may present with signs of elevated
intracranial pressure including depressed level of consciousness, decorticate or
decerebrate posturing, or flaccid areflexia.
Cerebellar Hemorrhage. The awake patient with cerebellar hemorrhage may complain
of severe headache, which may be localized to the suboccipital or upper cervical region.
Symptoms include those of cerebellar and lower brainstem dysfunction, as well as

59
hydrocephalus, which is frequently present. Signs of cerebellar dysfunction include
ipsilateral dysmetria, nystagmus when looking to the side of the lesion, and difficulties
with speech. Lower brainstem dysfunction typically manifests as difficulty with
swallowing. In the presence of hydrocephalus, signs of elevated intracranial pressure may
ensue. These include a decreased level of consciousness, decorticate or decerebrate
posturing, and flaccid areflexia.
Evaluation of Acute Headache (CT, MRI, and Lumbar Puncture).
In the patient who presents with the "worst headache of my life" a subarachnoid
hemorrhage should always be suspected. After initial resuscitation and complete
neurologic examination, head CT without contrast is mandatory. In addition to verifying
the presence or absence of subarachnoid blood, the CT scan should be carefully
scrutinized for other intracranial pathology, including hydrocephalus.
If the CT scan is positive for subarachnoid hemorrhage, the patient should undergo
cerebral arteriography. The arteriogram should be scrutinized for the location of the
aneurysm, as well as for any associated lesions including additional unruptured
aneurysms, arteriovenous malformations, and the presence or absence of vasospasm.
If the initial head CT is negative for subarachnoid hemorrhage, and the patient has a
history that is highly suspicious for subarachnoid hemorrhage, a lumbar puncture should
be performed. A small needle should be utilized and only a small volume of fluid should
be withdrawn. Non-clotting blood, xanthochromia, or a red blood cell count greater than
100,000 which does not drop significantly from the first to the last tube are all highly
suggestive of subarachnoid hemorrhage. If the lumbar puncture meets any of these
criteria, the patient should undergo arteriography as described above.
If the cerebral arteriogram is negative, in the face of a CT scan indicative of subarachnoid
hemorrhage or a positive lumbar puncture, the patient should be admitted to the hospital
for observation. At this point, an MRI scan of the brain with and without contrast should
be considered. MRI arteriography, if available, should be included. The purpose of this
study is to rule out other structural causes of subarachnoid hemorrhage including
angiographically occult vascular malformations and tumor. If no bleeding source is
identified with the MRI scan, most clinicians recommend repeating the arteriogram in 7
to 14 days. Occasionally, an intracranial aneurysm that was obscured by mass effect,
thrombosis, or vasospasm, may be detected.
Natural History and Broad Treatment Strategies of Intracranial Aneurysms and
Vascular Malformations.
Intracranial aneurysms - natural history.
Ruptured intracranial aneurysms.
In the patient who survives their initial aneurysm rupture, the most serious complication
is rebleeding. The rate of rebleeding is approximately 4% in the first 24 hours and
decreases to 1 to 2% per day for the first two weeks. After the first two weeks, the rate of
rebleeding drops to approximately 3% per year. A second potential serious complication
of aneurysmal subarachnoid hemorrhage is a vasospasm. Vasospasm is defined as
delayed narrowing of large and medium size arteries at the base of the brain. If severe,
this may lead to cerebral infarction. The risk of symptomatic cerebral vasospasm is
maximum between 4 and 11 days of subarachnoid hemorrhage. The incidence of
vasospasm, detected by angiography, is approximately 70%. The incidence of
symptomatic vasospasm (resulting in neurologic deficit) is approximately 25 to 30%. The

60
major risk factor for the development of vasospasm is the amount and location of
subarachnoid blood visualized on the CT scan. This is graded using the Fisher system;
Group Blood on CT Scan
1 No blood detected
2 Diffuse or vertical layers < 1mm thick
3 Localized clot and/or vertical layer >= 1mm thick
4 Intracerebral or intraventricular clot with diffuse or no SAH
Unruptured intracranial aneurysm - natural history.
Occasionally aneurysms are discovered prior to rupture. Again, the primary risk is
rupture. The available information suggests that the annual rate of rupture for an
unruptured intracranial aneurysm is between 1% and 3% per year. The risk of rupture is
affected by several factors. Larger aneurysms, particularly those greater than or equal to
10 mm in diameter, have a higher rate of rupture. Aneurysms located in the posterior
communicating artery, vertebrobasilar/posterior cerebral artery, and in the basilar tip are
more likely to rupture.
Treatment strategies for intracranial aneurysms.
The goal of intracranial aneurysm treatment is to prevent bleeding or rebleeding.
Additionally, in the patient with a ruptured intracranial aneurysm, treatment is also
instituted to lessen the risk of symptomatic vasospasm.
In patients with subarachnoid hemorrhage due to aneurysm rupture, outcome is related to
the modified Hunt-Hess grade. Higher-grade patients have a statistically poorer outcome.

Grade Description
0 Unruptured aneurysm
1 Asymptomatic, or mild headache and slight nuchal rigidity
No acute meningeal/brain reaction, but with fixed neurologic
1a
deficit
Cranial nerve palsy (e.g. III, IV), moderate to severe
2
headache, nuchal rigidity
3 Mild focal deficit, lethargy, or confusion
Stupor, moderate to severe hemiparesis, early decerebrate
4
rigidity
5 Deep coma, decerebrate rigidity, moribund appearance
*Add one grade for serious systemic disease (e.g. HTN, DM, severe
atherosclerosis, COPD), or severe vasospasm on arteriography
The patient with aneurysmal subarachnoid hemorrhage should be admitted to the
neurological intensive care unit. Adequate oxygenation and respiration should be assured.
The patient should be maintained normotensive. Hypertension should not be treated
unless the systolic blood pressure rises above 160 mm of mercury. Hydrocephalus, which

61
is present in at least 15% of patients with subarachnoid hemorrhage, should be treated
with cautious external ventricular drainage if symptomatic. Routine use of external
ventricular drainage is controversial. Cerebral selective calcium channel blockers lessen
the risk of symptomatic vasospasm and are started at this time. Use of anti-fibrinolytic
agents and corticosteroids may be considered.
Treatment of the ruptured intracranial aneurysm should at this point be entertained.
Direct surgical exclusion of the aneurysm from the circulation (craniotomy and clipping)
remains the "gold standard". Endovascular obliteration of the aneurysm through the use
of detachable coils may be considered in certain situations (difficult or "unclippable"
aneurysms, medically unstable patient). The long-term results of this modality of
treatment are unknown and currently under study.
Treatment of unruptured aneurysms takes into account both the risk of rupture, as well as
age of the patient, severity and progression of symptoms, and treatment alternatives.
Older patients have a statistically shorter life span and therefore lower cumulative risk of
rupture. Recent recommendations suggest that symptomatic intradural aneurysms of all
sizes should be considered for treatment. Unruptured aneurysms of all sizes in patients
with subarachnoid hemorrhage due to another treated aneurysm should be considered for
treatment. Treatment for unruptured small (less than 10 mm) aneurysms is not generally
recommended except in special circumstances. These include aneurysms near the 10 mm
size, those with daughter sac formation, and in patients with family history of aneurysm
or aneurysmal subarachnoid hemorrhage. Asymptomatic aneurysms greater than or equal
to 10 mm in diameter warrant strong consideration for treatment.
Arteriovenous Malformations - Natural History
Risks.
The primary risk of arteriovenous malformations is rupture. The rate of rupture has been
estimated to be approximately 4% per year for symptomatic arteriovenous malformations
and 2% per year for asymptomatic arteriovenous malformations. The rate of
arteriovenous malformation rupture appears to be higher in small malformations than in
large malformations. The second risk of arteriovenous malformation is seizures. Nearly
60% of patients with an arteriovenous malformation have a history of seizures. Larger
malformations are associated with a greater risk of preoperative seizures.
Treatment strategies for arteriovenous malformations.
Trnt for arteriovenous malformations is recommended when the risk of subsequent
hemorrhage is greater than the risk of treatment. The risk of treatment may be estimated
using the Spetzler-Martin classification:
Points
Graded Feature Assigned
Size of arteriovenous malformation
Small (<3cm) 1
Medium (3-6cm) 2
Large (>6cm) 3
Eloquence of adjacent brain
Noneloquent 0

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Eloquent 1
Pattern of venous drainage 0
Grade = [size] + [eloquence] + [venous drainage]

There are several treatment options for arteriovenous malformations. These include
craniotomy and direct surgical excision, embolization using interventional radiologic
techniques, and Gamma Knife radiosurgery. Because the risk of treatment rises with
increasing Spetzler-Martin grade, the following recommendations have been made:
Grade I or II arteriovenous malformations should be treated. Grade III lesions should be
treated if symptomatic. Grade IV or V arteriovenous malformations should be treated
only when significant or repetitive intracerebral hemorrhage has occurred or the patient is
experiencing progressive neurologic disability. The treatment modality of choice remains
controversial. Surgical removal remains the "gold standard". With all treatment
modalities, the goal remains complete removal or obliteration of the arteriovenous
malformation. This goal should be pursued until it has been achieved.
Migraine, Cluster, and Tension Headache, and Sinusitis Headache.
Migraine headache. Features of classical migraine headache include:
Onset in childhood, adolescence or early adulthood, positive family history, more
common in females, usually unilateral, throbbing pain, nausea and vomiting common,
scintillating scotomata, duration of several hours.
Cluster headache. Much more common in men, pain severe and retro-orbital or
temporal, usually unilateral, pain at night, accompanied by lacrimation, rhinorrhea,
periorbital edema, duration of pain 20 to 60 minutes and may recur several times within a
24-hour period. Pain-free period of months to years is typical.
Tension headache. Onset in adolescence or young adulthood, non-familial, pain is
typically bilateral, bifrontal, bitemporal, or suboccipital, pain described as throbbing or a
sensation of "pressure", nausea and vomiting rare, no lacrimation or rhinorrhea, more
frequent in afternoon or evening, duration hours to days. precipitated by stress,
depression, or anxiety.
Sinusitis. History of allergic background or frequent prior sinusitis, unilateral or bilateral,
localized pain and tenderness over affected sinuses common, associated conditions
include nasal obstruction, fevers, and chills, history of scintillating scotomata, nausea, or
vomiting. Headache persists until sinusitis is cleared.
Diagnosis and Management of Ischemic Cerebrovascular Disease
Knowledge of the symptoms and signs of occlusive cerebrovascular disease is necessary
for effective diagnosis and management of these patients. Symptoms are often transient,
may be subtle, and can be unappreciated by both patient and physician. As most
occlusive cerebrovascular is secondary to atherosclerosis of the internal carotid artery, the
following brief review will focus on patients with this particular problem.
Transient Ischemic Attack
Transient ischemic attack (TIA), a transient neurological deficit secondary to dysfunction
of a part of the cerebral hemisphere because of a lack of blood flow, is the most common
symptom in patients with atherosclerotic carotid artery disease. TIA has been
traditionally defined as lasting less than 24 hours.
Vague symptoms such as dizziness, confusion and blurred vision are not TIAs. Unilateral

63
weakness, clumsiness and numbness are the most common symptoms but dysphasia and
dysarthria may also occur. The diagnosis is based on a careful history, as most patients
will not have discernible neurological deficits when being examined. Many conditions,
including seizures, intracranial tumors and hematomas, cardiac dysrhythmias,
hypoglycemia and migraine may produce symptoms similar to TIAs. These other
etiologies must be considered in a patient suspected of having TIAs.
Amaurosis Fugax
Amaurosis fugax (AF), a transient loss of vision involving one eye, is the second most
common presenting symptom in patients with atherosclerotic carotid artery disease. This
can be confused with a transient visual problem involving both eyes in one visual field
and careful questioning will be necessary to differentiate between the two. Many patients
will not have tested each eye separately during an episode of visual loss, in which case
differentiation may be impossible.
Evaluation of atherosclerotic Carotid Artery Disease
General
As indicated above, patients with suspected carotid artery stenosis need a careful history
to identify symptoms suggestive of AF or TIA and to assess their risk factors for
generalized atherosclerotic vascular disease. Such risk factors include advanced age,
smoking, diabetes, hypertension, hyperlipidemia, peripheral vascular occlusive disease,
coronary artery disease and a positive family history of stroke or myocardial infarction.
General physical and neurological examinations looking for evidence of peripheral and
cerebrovascular arterial stenosis or neurological deficit is done following a careful
medical history. The presence of a cervical bruit on auscultation of the neck may indicate
carotid artery stenosis producing turbulent blood flow. This is the most common physical
finding in patients with clinically significant atherosclerotic carotid artery disease but its
absence does not rule out the diagnosis. Diminished peripheral pulses may indicate
generalized atherosclerotic vascular disease.
Fundoscopic Examination
Cholesterol emboli may occasionally be seen in retinal vessels of patients with
atherosclerotic carotid artery disease with or without AF.
Laboratory Evaluation
Patients with TIAs should have a study such as a cranial CT or MRI scan to rule out
intracranial lesions. Imaging of the carotid arterial system with carotid duplex
ultrasonography, magnetic resonance angiography, CT angiography or digital subtraction
angiography should also be done if atherosclerotic carotid artery disease is suspected
from the history and/or examination. Echocardiography may also be indicated to look for
a cardiac source of embolism. Screening laboratory evaluation to look for evidence of
coagulopathy (PT, PTT, INR), vasculitis (ESR) or hyperlipidemia should also be
considered.
Indications for Carotid Endarterectomy
Patients with symptomatic atherosclerotic carotid artery disease producing a 70% or
greater diameter stenosis of the internal carotid artery disease benefit from surgery if the
perioperative morbidity and mortality is less than 7%. Patients with asymptomatic disease
and greater than 60% stenosis benefit from surgery if perioperative complications are less
than 3%. If in doubt as to the potential benefit of carotid endarterectomy patients should
be referred to skilled cerebrovascular surgeon for evaluation.

64
6. DIAGNOSIS AND MANAGEMENT OF SPINAL CORD INJURY.
Mechanism of Injury:
Motor vehicle crashes: 45%
Falls: 22%
Sports: 14%
Violence: 14%
Other: 5%
Most patients (75%) are males. Sixty percent are between the ages of 16 and 30. Slightly
more than half of all patients with cervical spine trauma will present with signs of
neurologic injury. Up to 10% of patients with cervical spine trauma will develop signs of
neurologic injury after presentation.
Association with other injuries
Five to ten percent of unconscious patients who have been involved in a fall or motor
vehicle accident will have a cervical spine injury. Conversely, 60% of patients with a
cervical spine fracture will have at least one major associated injury. Five to fifteen
percent of patients with a cervical spine fracture will have a second vertebral column
fracture.
History and physical examination clues to the presence of spinal injury
Pain
local pain, due to bone/soft tissue injury
radicular pain, due to nerve root compression
bradycardia indicating loss of sympathetic input to the heart from cervical or high
thoracic lesion
hypotension due to loss of sympathetic input to systemic vasculature ("spinal shock")
External tenderness, bruising, or swelling due to local soft tissue damage
palpable step-off due to malalignment
paraspinous muscle spasm
torticollis due to muscle spasm or unreducedvertebral dislocation
Weakness partial vs. complete
level: motor level is defined as the most caudal level with grade III (antigravity) strength,
assuming that more cranial levels have grade IV strength
Sensation decreased vs. absent vs. hyperesthesia
level : sensory level is defined as the most caudal dermatome with normal sensation
saddle region needs to be tested - may be the only region of preserved sensation ("sacral
sparing")
Reflexes
normal vs. absent vs. increased
absent may indicate the presence of spinal shock
increased may indicate the presence of an older complete or incomplete injury
Sphincter
decreased or absent tone
loss of voluntary contraction
loss of bulbocavernosis reflex
In the comatose or intoxicated patient
The comatose or intoxicated may be unable to transmit information regarding pain or
cooperate with strength or sensory testing. Intracranial injury may co-exist with spinal

65
injury. Therefore one must assume that all comatose or intoxicated trauma patients have a
spinal injury until proven otherwise.
Clues to the presence of a spinal injury in such a patient include:
flaccid areflexia
diaphragmatic breathing
loss of grimace to painful stimuli below specific level
loss of withdrawal response below a specific level
loss of spontaneous movement below a specific level
loss of sphincter tone
priapism
Frankel Grading System.
Frankel Grade Designation Definition
Complete
No motor or sensory function detected below
neurological A
level of lesion
injury
No motor function detected below level of
Preserved
B lesion, some sensory function below level of
sensation only
lesion preserved
Some voluntary motor function preserved
Preserved motor, below level of lesion but too weak to serve any
C
nonfunctional useful purpose, sensation may or may not be
preserved
Preserved motor, Functionally useful voluntary motor function
D
functional below level of injury is preserved
Normal motor Normal motor and sensory function below
E
function level of lesion, abnormal reflexes may persist

Radiologic studies
Plain x-rays
Static:
Need to see down to the C7-T1 disc space on the lateral plain x-rayof the cervical spine
Anterior-Posterior and Lateral (Cervical/Thoracic/Lumbar)
Evaluate for the presence of:
Prevertebral soft tissue swelling
Malalignment
Vertebral body fractures or loss of vertebral body height
Posterior element fractures (spinous process, transverse process, lamina, facet)
Widening of interpedicular distance
Fracture of dens
Open Mouth Odontoid (Cervical)
Evaluate for the presence of:
Fracture of dens
Widening of lateral masses of C1
Dynamic (Flexion/Extension):

66
Performed only if static x-ray are normal but there is a high suspicion of ligamentous
injury not be visible on static x-rays

Can only be performed in the conscious cooperative patient


Evaluate for the presence of:
Vertebral subluxation
Widening of distance between dens and arch of C1
Abnormal kyphosis or opening up of posterior elements in flexion
Computed axial tomography
Has largely replaced conventional polytomography
Most useful for assessing bone detail; poor visualization of neural elements
Usually performed whenever the plain x-rays are abnormal in order to more completely
characterize a known fracture, rule out spinal canal compromise due to indriven bone
fragments with a known fracture, rule out occult fracture in the presence of known
ligamentous injury.
Used to image the lower cervical spine when this cannot be done using plain x-rays
May be combined with intrathecal contrast (myelography) in order to visualize neural
elements when MRI is contraindicated or not available
Magnetic resonance imaging (MRI)
Most useful for visualizing soft tissue structures
Resolution of bone inferior to computed axial tomography
May be performed when plain x-rays are abnormal in order to rule out: spinal cord
injury/hematomyelia, traumatic intervertebral disc herniation, hematoma, ligamentous
injury.
Acute management of spinal cord injury
Immobilization
Cervical.
Traumatic cervical spine injury should be presumed to be present until it is ruled out by
physical examination and appropriate radiographic studies. In order to prevent new or
additional neurologic injury, immobilization of the cervical spine should be instituted as
soon as possible. Patients with a known or suspected cervical spine injury, or those who
are comatose or intoxicated at the scene of injury, should ideally be placed in a cervical
orthosis at the scene. Alternatively, if an appropriate cervical orthosis is not available, the
head and neck may be immobilized by placing sandbags or towel rolls on either side of
the head.
If a cervical spine injury is found radiographically, it is usually imperative that cervical
spine immobilization be continued until definitive management is complete. In some
patients this may consist of continued treatment in a cervical orthosis, combined with bed
rest.
In other patients, especially those with neurologic injury, cervical spinal instability, or
unreduced cervical spinal dislocation, in-line cervical traction may be used. Cervical
traction is frequently applied using Gardner-Wells tongs secured to the skull. Five pounds
of weight are initially applied. More weight is occasionally added in an attempt to reduce
a spinal dislocation. While the patient is in cervical traction, neurologic examinations
should be performed frequently. Radiographic examinations should be performed after
the initial application of weight and after any subsequent change to assess for changes in

67
spinal alignment and the presence of overdistraction (pulling apart of the spine). Weight
should be immediately reduced if a decline in the neurologic examination or
overdistraction occurs. Cervical traction is contraindicated in certain highly unstable
cervical injuries, as well as in occipital-cervical dislocation.
Finally, some patients may initially be placed in a halo vest. In addition to effective
immobilization of the upper and middle cervical spine, placement in a halo vest may
constitute definitive therapy for a variety of lesions.
Thoracolumbar
As with the cervical spine, thoracolumbar spine fracture should be presumed to be
present until it is ruled out by physical examination and appropriate radiographic studies.
Present extrication and transport techniques emphasize maintenance of a neutral position
during the prehospital phase. Most commonly, patients are transported to the hospital on
a backboard. They should not be allowed to sit or stand prior to evaluation. During the
physical examination, patients should be carefully logrolled by multiple personnel for
examination of the back. Unless the patient has a highly unstable lesion, the backboard
may be removed while the patient is flat in bed. Whenever the patient is moved, for
example to the CT or MRI scanner, the patient should be carefully placed back onto the
backboard.
Steroids in Spinal cord injury
Results of the NASCIS II study showed that patients with spinal cord injury who were
treated with methylprednisolone within eight hours of injury had significantly greater
improvement in their neurologic function (motor, pinprick sensation, and touch
sensation) than those given a placebo. The dose administered in the study was 30mg/kg
given as a bolus over 15 minutes, followed by an infusion of 5.4 mg/kg/hr for 23 hours,
begun 45 minutes after completion of the bolus.
Results of a follow-up study, NASCIS III, concluded that patients with acute spinal cord
injury who receive methylprednisolone within 3 hours of injury should be maintained on
the treatment regimen for 24 hours. When methylprednisolone is initiated 3 to 8 hours
after injury, patients should be maintained on steroid therapy for 48 hours.
Gunshot wounds to the spinal cord
No data from randomized, controlled studies. However, a recent retrospective study of
254 patients reported no neurological benefit from intravenously administered steroids
after a gunshot wound to the spine (C1-L1). Both infectious and noninfectious
complications were more frequent in the group receiving steroids. It was recommended
that patients with spinal cord injury secondary to a gunshot wound to the spine not be
treated with steroids.
Respiratory
Maintenance of an adequate airway and breathing remains the first priority in the trauma
patient. Spinal cord injured patients may suffer from inadequate respiratory function due
to paralysis of the intercostal muscles or diaphragm. Concomitant injuries may also
compromise respiratory function. Maintenance of spinal alignment is critical during
intubation. If endotracheal intubation is needed, it is best performed in conjunction with
in-line cervical traction, nasotracheally (if there is no evidence of basilar skull fracture) or
fiberoptically.
Cardiac
After airway and breathing abnormalities are treated, adequate perfusion must be assured.

68
Hypotension in the patient with a spinal injury may be due to intravascular volume loss
due to bleeding or spinal cord injury. These two processes may co-exist. Identification
and control of bleeding is the first priority. Hypotension, due to loss of sympathetic
vascular tone, and bradycardia, due to loss of sympathetic innervation of the heart, are the
most important elements of spinal shock due to spinal cord injury. Initial treatment of
spinal shock consists of intravascular volume expansion. In patients refractory to this
therapy, sympathomimetic drugs may be needed
Genitourinary
Placement of an indwelling urinary catheter is mandatory in the severely injured patient
for monitoring of urine output volume. In the patient with a spinal injury, urinary
catheterization during the period between identification of the injury and definitive
treatment facilitates nursing care and avoids unnecessary patient movement. In the
neurologically injured patient, the bladder may not function normally. Continuous urinary
drainage prevents the complication of bladder rupture due to overdistension.
Management principles of the unstable spine
Unstable spine: Definition
Spinal instability has been defined as "the loss of the ability of the spine under
physiologic loads to maintain relationships between vertebrae in such a way that there is
neither damage nor subsequent irritation to the spinal cord or nerve roots and , in
addition, there is no development of incapacitating deformity or pain due to structural
changes." Notice that this is a clinical definition.
Numerous sets of radiographic criteria have been developed in an attempt to predict
which patients are or will become unstable after a spinal injury. The most commonly
used is the three-column model of Denis. In this model, the spine is divided into a
posterior, middle, and anterior column. The posterior column includes all of the posterior
bony and ligamentous elements while the middle column includes the posterior
longitudinal ligament and all of the elements comprising the posterior one third of the
vertebral body and intervertebral disc. The anterior column is comprised of the remaining
portions of the vertebral body and intervertebral disc, as well as the anterior longitudinal
ligament. Injuries with incompetence of two or three columns are inferred to be unstable.
The three-column theory applies to the thoracolumbar spine only.
Management principles
As described above, initial management of the unstable spine consists of immobilization
of the injured vertebral segment while the patient is being stabilized and other injuries are
being ruled out. After this initial phase, management decisions are generally based on
three factors:
Need for decompression of neural elements.
General indications for neural decompression will be discussed in the next section. It is
important to consider that, in some cases, decompressive procedures may further
destabilize the unstable spine or render the stable spine potentially unstable.
Need to mobilize the patient as soon as possible.
Some unstable spinal injuries may be potentially treatable with prolonged bed rest.
However, by avoiding prolonged periods of bed rest, early surgical stabilization of the
unstable spine may help to prevent atelectasis, pneumonia, deep venous thrombosis, and
decubiti. In addition, early stabilization allows the patient to begin rehabilitation earlier,

69
potentially reducing the complications of joint contracture and deconditioning, as well as
potentially reducing time off work.
Need to stabilize the spine that is not likely to heal without surgical intervention.
In general, injuries that are not likely to heal include those with widely displaced
fractures, unreduced or unreducible dislocations, severe deformity, or severe ligamentous
injury. Also included in this group are injuries which have been treated with prolonged
bed rest or bracing which have not healed correctly.
Surgical stabilization has two components. The first component is arthrodesis, or fusion.
The goal of fusion is to induce adjacent vertebrae above and below the injury to heal
together into a solid block of bone, eliminating any potential movement between them.
This usually involves placement of bone graft between the vertebrae. Bone graft may be
placed anteriorly, between adjacent vertebral bodies, or posteriorly, between adjacent
laminae, facets, or transverse processes.
The second component of surgical stabilization involves internal fixation
(instrumentation). This provides immediate strength and maintains anatomic alignment
during the time it takes for fusion to occur. Internal fixation usually involves the
implantation of some combination of wire, hooks, screws, and/or rods. Internal fixation is
not a substitute for fusion. A general principle is that all internal fixators will eventually
fail if fusion does not occur.
Management principles in spinal cord injury.
Indications for surgery
The most compelling indication for decompressive surgery in spinal cord injury is the
presence of an incomplete neurologic injury with persistent neural compression at the site
of injury. Compression may be due to indriven bone fragments, traumatic disc herniation,
epidural hematoma, or persistent vertebral malalignment. While there is currently debate
regarding the most appropriate timing of decompression in the presence of a stable
neurologic examination, most clinicians agree that it should be done emergently in the
presence of a rapidly declining neurologic examination.
The goal of decompressive surgery is restoration of a normal spinal canal without
additional injury to the neural elements. This, in theory, will facilitate neurologic
recovery. A review of the most common causes of neural compression reveals that most
are ventral processes. Therefore, technically demanding ventral decompressive
procedures are frequently necessary. As has been previously stated, these procedures may
further destabilize the unstable spine or render the stable spine potentially unstable.
Treatment of medical complications associated with cord injury
Skin:
Patients with spinal cord injury are at particular risk for skin breakdown due to inability
to sense pain and prolonged periods of bed rest. This may be further aggravated by
incontinence and the necessity of wearing an orthosis. All insensate regions should be
surveyed regularly. While in bed, spinal cord injured patients should be turned every two
hours. Bony prominences (sacrum, heels) should be protected. Shearing forces, which
frequently occur during transfers, should be avoided. Bowel and bladder dysfunction
should be treated accordingly as described below. All orthoses and wheel chairs should
be fitted appropriately and adjusted to accommodate weight loss or gain.
Bladder:
Patients with spinal cord injury frequently suffer from some form of urinary bladder

70
dysfunction. This may manifest as a lower motor neuron bladder with overflow
incontinence and urinary retention. Alternatively, an upper motor neuron bladder may be
present with incontinence and reduced bladder capacity. In either instance, the goals of
treatment are to maintain a functional lower urinary tract free of infection and to preserve
renal function. As has been described above, the initial treatment of urinary bladder
dysfunction is usually placement of an indwelling urinary catheter. After the acute period,
sterile, intermittent catheterization is preferable to the long-term use of an indwelling
urinary catheter. Intermittent catheterization should initially be performed every 4 hours
and urinary volumes should not be permitted to exceed 450cc. If possible, patients may
be taught to perform self-catheterization. Renal function should be closely monitored
long-term.
Bowel movement:
Patients with spinal cord injury also frequently suffer from some form of bowel
dysfunction. The goals of management are for the patient to be free of incontinence and
to develop a predictable bowel routine without fecal impaction. Patients with lower motor
neuron bowels frequently require manual removal of feces. Those with upper motor
neuron bowels may require daily digital rectal stimulation or suppositories. Adequate
daily fluids and fiber intake facilitate development of a bowel routine. Diarrhea may be
due either to excessive laxative intake or fecal impaction.
Respiratory:
All trauma patients are susceptible to atelectasis and pneumonia during the acute phase.
This is especially true in the patient with concomitant traumatic brain injury. Incentive
spirometry should be instituted early in treatment. If the patient is not able to participate,
consideration should be given to the use of intermittent positive pressure breathing
(IPPB).
Additional problems arise in the spinal cord injured patient. The phrenic nerve is supplied
by the C3, C4, and C5 roots. Patients with a C4 or higher level of injury most often
require placement of a tracheostomy and the assistance of a ventilator. Patients with a C6
or higher level of injury will require some assistance with cough and clearing of
secretions. Patients with a low cervical or thoracic level of injury remain at higher risk for
atelectasis, pneumonia, and respiratory failure, despite normal phrenic nerve function,
due to dysfunction of the intercostal musculature. All should be started on incentive
spirometry, as well as exercise programs to increase strength in the remaining muscles of
inspiration. Prophylactic clearing of secretions should be encouraged.

7. DIAGNOSIS AND MANAGEMENT OF HYDROCEPHALUS AND SPINAL


DYSRAPHISM
Hydrocephalus
Definition of acute and chronic hydrocephalus:
Hydrocephalus is the imbalance between spinal fluid production and absorption leading
to a build-up of cerebrospinal fluid (CSF).
Normal physiology and pathophysiology of hydrocephalus:
Cerebrospinal fluid (CSF) is a liquid that normally surrounds the brain and spinal cord,
serving to cushion the nervous tissue as well as wash away metabolic byproducts. It
probably serves other functions, which are not as well understood. CSF is made within
the brain by choroid plexus. This specialized modified cuboidal epithelium secretes CSF

71
at a constant rate of .37 cc/minute, or roughly 500 ccs per day. The CSF production rate
is energy dependent and constant. The composition of this fluid is similar to an
ultrafiltrate of plasma. Choroid plexus in found within the 4 ventricles of the brain, the
two lateral ventricles, the midline third ventricle and the fourth ventricle of the posterior
fossa. The CSF normally circulates through these ventricles by way of communication
pathways. The two lateral ventricles join the third ventricle by way of the Foramena of
Monro. The third ventricle joins the fourth ventricle by way of the Aqueduct of Sylvius,
and CSF exits the fourth ventricle by way of the midline foramen of Magendie and the
lateral Foramina of Luschka. CSF then circulates up over the convexity of the cerebral
hemispheres where it is absorbed by another specialized tissue, the arachnoid
granulations. The arachnoid granulations are a single cell layer of cuboidal epithelium
which allow CSF to cross into the venous system in an energy passive process by forcing
fluid across the membrane, utilizing the pressure differential between the intracranial
pressure (ICP) and the pressure within the venous sinuses. Normally, the arachnoid
granulations can absorb several times more than that which is produced; however, if the
arachnoid granulations become scarred because of trauma or infection, or if they become
obstructed by blood products from a hemorrhage, then CSF can no longer be absorbed
well and begins to build up in the subarachnoid spaces, putting pressure on the brain. If
CSF flow is blocked at this level, this is termed "communicating hydrocephalus". If the
CSF pathways are blocked elsewhere, such as at the level of the aqueduct of Silvius, then
this is termed obstructive hydrocephalus. As the intracranial pressure begins to rise in
response to a build up of CSF, the patient becomes symptomatic.
Symptoms of hydrocephalus
Acute:
If the blockage of CSF flow happens rapidly, such as following a sudden hemorrhage, the
brain has little time to compensate and the intracranial pressure rises rapidly and to a
sufficient degree to cause rapid deterioration into coma. Patients will typically experience
headache, nausea and vomiting, followed in a period of a few hours by confusion,
agitation and then somnolence. If acute hydrocephalus is not treated emergently, the
patient’s intracranial pressure will reach the point at which cerebral perfusion is
compromised and the patient will deteriorate from coma to death.
Chronic:
If there is partial obstruction or partial blockage to CSF absorption such that CSF
pressure builds gradually, then the brain accommodates to this change at first, and the
onset of symptoms is more insidious. This is typically seen in patients with slow growing
tumors. They typically develop headaches and nausea at night or upon awakening in the
morning, with improvement in their symptoms as they get up and walk around. In infants
with open sutures, chronic hydrocephalus manifests by increased head growth, which
crosses percentiles on the growth curve.
Signs of hydrocephalus
Acute:
In the young child with an open fontanelle, an infant will develop a bulging fontanelle
and separation of the cranial sutures. Rapid head growth is noted in premature and
newborn infants. These young infants may also have intermittent episodes of bradycardia
and apnea. They may develop crossing of the eyes in response to stretching of the
abducens nerves or may develop a conjugate downgaze, termed the "setting sun" sign. In

72
the young infant, irritability and somnolence is a late occurrence.
In older children and adults, the development of papilledema occurs over hours to days
and may not be seen in the acute state. Severe headache, vomiting and alteration of
mental status are followed by somnolence as cortical perfusion becomes further
compromised.
Chronic:
Chronic hydrocephalus beyond infancy often manifests similarly to the syndrome of
Normal Pressure Hydrocephalus in which the patient presents with an ataxic gait, urinary
incontinence, and either dementia or a decline in short-term memory. Patients may
describe intermittent headaches which are typically worse upon arising first thing in the
morning or which awaken them from sleep at night. They may have chronic papilledema
or optic atrophy with constriction of their visual fields.
Radiographic diagnosis of hydrocephalus
Skull x-ray:
Skull radiographs can demonstrate findings of raised intracranial pressure, are
inexpensive, and are readily available in most physicians’ offices. Separation of the
cranial sutures, demineralization of the sella turcica, or a J-shaped sella may indicate
chronically raised intracranial pressure.
Ultrasound:
In the newborn infant with an open fontanelle, sonography at the bedside can demonstrate
the ventricular size and large subdural collections. Small subdurals can be missed.
Insonation through the mastoid can image the posterior fossa and rule out 4th ventricular
masses.
Computerized tomography:
This is the imaging modality of choice for screening for hydrocephalus. It is relatively
inexpensive and is of sufficient detail to rule out most tumors which might obstruct the
ventricular system.
Magnetic resonance imaging:
MRI gives the highest resolution image of the brain. It has the advantage of multiplanar
imaging, which can be useful in determining subtleties such as agenesis of the Foramen
of Monro or aqueductal stenosis. With resolution down to 0.5mm, a MRI is unlikely to
miss even the smallest of tumors.
Differential diagnosis of hydrocephalus
Acute:
The differential diagnosis of acute hydrocephalus is age dependent. In the premature
infant, it will most commonly be secondary to a spontaneous intraventricular
hemorrhage. In the newborn, it may be secondary to a congenital abnormality of the CSF
pathways, may be secondary to neonatal meningitis, or may be caused by a congenital
brain tumor.
Chronic:
Chronic hydrocephalus is caused by a slowly growing brain tumor until proven
otherwise. It may be secondary to a congenital abnormality such as aqueductal stenosis,
or possibly to one of the chronic meningitides, but CT or MRI must rule out a mass lesion
first.
Treatment of hydrocephalus:
The treatment of hydrocephalus is dependent upon its cause. Acute hydrocephalus

73
secondary to hemorrhage or infection is often transient and can be managed by temporary
CSF drainage, either by serial lumbar punctures (LPs) or by placement of a temporary
ventricular drain until the underlying pathology has been dealt with. Chronic
hydrocephalus has classically been treated by a shunt, which is a plastic tube and valve
system which offers a manmade plumbing system to replace the natural one which is no
longer working. This most commonly involves placement of a tube into the ventricle
which exits the skull through a drilled hole (burr hole) and is connected to a one way
pressure regulating valve and then a distal tube which drains excess fluid into another
body cavity where it can be absorbed. Most often, this is the peritoneum.
Spina Bifida
Definition of Spina Bifida:

Spina bifida is a developmental abnormality in which there is incomplete fusion of the


dorsal elements composing the roof of the spinal canal. If this birth defect is skin
covered, it is termed "occulta". If the defect is open and the neural elements are exposed,
it is termed "cystica" or "aperta".
Normal development and abnormal development related to spina bifida:
In the course of normal development, the human embryo at day 18 of gestation is
composed of 3 primordial layers of tissue, the ectoderm, the mesoderm, and the
endoderm. Shortly thereafter, the ectoderm begins to develop two raised areas, one on
either side of the primitive streak. These folds of tissue comprise the neural crest tissue,
which curves together and fuses across the midline. This fusion expands in both the
rostral and caudal directions to form the neural tube. The anterior neuropore closes at
around day 24 of life and the posterior neuropore around day 28. Failure of closure of the
anterior neuropore will cause anencephaly, whereas failure of closure of the posterior
neuropore is associated with spina bifida cystica.
Signs of spina bifida
Open:
Spina bifida cystica of aperta is being increasingly diagnosed by prenatal ultrasound.
When not diagnosed prenatally, it generally becomes readily apparent at birth, with the
fetus being born with a large head and a myelomeningocele. This condition is usually
associated with additional abnormalities such as pes cavus deformity of the feet and
neurogenic bowel and bladder.
Closed:
Spina bifida occulta can be a variant of normal, with 5% of the population demonstrating
incomplete fusion of the neural arches on spine x-ray. Most of the time, this is not
associated with neural abnormalities. At times, the incomplete arch is accompanied by
other midline lumbar ectodermal abnormalities. These include an abnormal pit in the
skin, representing a rudimentary sinus tract, an abnormal lipomatous collection, a tuft of
hair, or an area of cutis aplasia (abnormal skin similar to a birth mark). When found on
screening physical exam, this should alert the physician to the possibility of an
underlying dysraphism. The filum terminale is the terminal extension of the pia of the
spinal cord. It forms a small linear structure, which normally connects the end of the
conus medullaris to the dura at the end of the thecal sac. In the fetus, the spinal cord
extends to the end of the sacral spinal canal, but over time, there exists a differential rate
of growth between the vertebrae and the neural elements such that the end of the spinal

74
cord migrates rostrally within the spinal canal. At birth, the end of the conus in normally
around L3, and by six months of age, it is normally between T12 and L2. In cases of
abnormal development, a thickened filum terminale, a spinal lipoma, or the bony spicule
associated with diastematomyelia may serve to tether the spinal cord and prevent the
normal rostral migration. This tethering will lead to progressive dysfunction of the distal
spinal cord, that which controls bowel and bladder function, sexual function, and distal
lower extremity function.
Symptoms of spina bifida
Open:
Spina bifida aperta is generally too obvious to be symptomatic. The infant has deformity
of the lower extremities, an enlarged head circumference, and a neural tube defect, which
is obvious. In some instance, these infants may develop symptoms of hindbrain
compression secondary to a Chiari malformation. This malformation is commonly found
in patients with spina bifida aperta and results in their brainstem structures being in their
cervical spinal canal. Chiari symptoms at this age may consist of drooling, feeding
difficulties, a hoarse or high pitched cry, vocal cord paralysis or other signs of lower
cranial nerve dysfunction.
Closed:
The symptomatic spina bifida occulta typically becomes symptomatic beyond infancy,
typically following a growth spurt. Classically, the young child who has become toilet
trained begins to experience urinary incontinence and urgency. This is often accompanied
by back pain exacerbated by exercise, similar to the syndrome of lumbar
pseudoclaudication seen in elderly adults. Numbness of the legs, dysesthesias in the
lower extremities and motor symptoms can also be seen. Most of these children will have
a history of chronic constipation.
Radiographic diagnosis of spina bifida
Plain radiographs:
Plain radiographs of the spine are generally diagnostic. Occasionally, more subtle
abnormalities will be found such as the midline upper lumbar calcification of
diastematomyelia or widening of the pedicles from a chronic intraspinal mass such as a
spinal arachnoid cyst.
Ultrasound:
In the newborn period, the dorsal arches of the spine are cartilaginous and have yet to
ossify. In the first few weeks of life, ultrasound over the distal spine can adequately
image the spinal canal and cord to determine the level of the conus medullaris, detect
intraspinal lipomas and fluid collections such as syringomyelia. At this age, a thickened
filum terminale can also be detected.
CT and CT myelography:
The CT scan is an excellent mode of imaging abnormal bone anatomy and is important in
defining abnormal segmentation defects such as butterfly vertebrae or diastematomyelia.
In the MRI era, myelography is only rarely performed in the child.
MRI:
This is the imaging modality of choice for defining abnormalities of the neural elements
associated with spina bifida occulta or tethering of the spinal cord. In the newborn period
MRI may be difficult due to respiratory and cardiac motion artifact. If the infant is

75
clinically stable, most pediatric neurosurgeons prefer to wait until the infant is 3-6
months old to perform this study.
Treatment:
In the infant born with a myelomeningocele, repair is usually performed within the first
48 hours of life. The early repair of a leaking myelomeningocele is believed to prevent
the development of meningitis. Ninety percent of these infants will also have
hydrocephalus and will require placement of a ventriculo-peritoneal (VP) shunt. The
repaired myelomeningocele always scars into the walls of the spinal canal at the site of
repair; therefore, all of these infants have, by definition, a tethered spinal cord. Over time,
at least a fourth of children with spina bifida will become symptomatic from this
tethering and will require further surgeries to untether the spinal cord.
In the asymptomatic infant noted to have spina bifida occulta detected by a midline
lumbar cutaneous signature mark, treatment continues to be controversial. Most pediatric
neurosurgeons will untether the asymptomatic infant due to reports of progressive neural
dysfunction noted in many infants, which are followed over time without treatment.
Given that all neonates are incontinent, it is difficult to assess bowel and bladder function
at this age, and once lost, surgical intervention cannot reliably restore function, but can
only halt the deterioration; therefore, adequate evidence exists to support prophylactic
untethering.
8. DIAGNOSIS AND MANAGEMENT OF NON-TRAUMATIC NECK AND
BACK PROBLEMS.
Categories of Symptoms:
In order to have an orderly approach to the patient with spine related complaints, the
physician needs to be familiar with the broad categories of symptoms with which patients
will present. The differential diagnosis of spine related pain is extensive. Although the
vast majority of patients have benign, non-surgical problems, very serious medical illness
can present with the chief complaint of back, neck, or extremity pain. With experience,
common presenting syndromes emerge, and allow more rational triage of patients into
groups who need further evaluation, and perhaps surgical referral, and those who are best
managed without extensive testing and referrals.
Axial Spine Pain
Pain in the spine with or without radiation into the extremities is the most common
presenting compliant related to spinal disease. Initial evaluation should center on
obtaining a detailed history of the patient’s complaints and previous medical history. A
history of recent trauma should raise the suspicion of a fracture. Character, severity,
frequency, inciting and relieving events should be thoroughly explored. Inquiry about
associated symptoms, such as fever and visceral pain can suggest diagnoses and avenues
of investigation. The timing of pain can also lead to appropriate investigation. Morning
stiffness and pain that relents as the day progresses suggests an inflammatory disorder,
where nocturnal pain associated with recumbency is a much more ominous symptom,
being seen with malignant, destructive lesions.
Activity-related back pain without neurologic symptoms or findings is quite common. In
the absence of other worrisome symptoms or findings, most patients can be successfully
managed with a brief reduction in activity level and analgesics. The optimum duration
and content of non-operative treatment is controversial. Some practitioners advocate
strict bed rest, while others avoid it. Reduction of general activity level while avoiding

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any specific inciting events, coupled with non-steroidal agents, is the mainstay of most
clinicians’ first line therapy.
Evaluation for pure axial spine pain that does not resolve with conservative measures can
include plain radiographs, magnetic resonance imaging, computed tomography,
myelography, electrophysiologic studies, and discography. A set of plain films can
exclude fracture, and identify serious bony pathology such as a destructive tumor.
However, for soft tissue imaging the MRI is the study of choice. CT can give detailed
information about bony anatomy. Myelography is sensitive to canal and foraminal
pathology, and when paired with a post-myelogram CT scan, can be very useful in the
patient in whom an MRI is not an option. Discography is controversial, and will not be
discussed at length.
Radiculopathy
Referred pain into the upper or lower extremities often accompanies back or neck pain.
Referred pain can be the initial symptom of a compressed nerve root by a ruptured disc or
neural foraminal stenosis from osteophytes. Radicular pain is usually described as sharp
or even shock-like, and may be associated with certain activities or positions. The
distribution of the pain may not always be classic, and often doesn’t respect dermatomal
distributions.
Sensory changes are also often seen, with complaints of tingling and numbness being
very common. On examination decreased sensation to pinprick and light touch are found
in a dermatomal distribution in many patients. It is interesting that areas of referred pain
and sensory loss often are different. Making determinations of level of nerve root
compression solely from pain or sensory distribution is often difficult.
Motor weakness is also seen in nerve root compression syndromes. Muscle innervation is
more constant and has less overlap than sensory innervation and is better at predicting
level of pathology. Motor deficits that are of a more long-standing nature can have
significant wasting. Hyporeflexia in the appropriate distribution is also seen.
Cervical
Cervical radiculopathy can present acutely, as with a traumatic ruptured disc, or can be of
a more chronic and intermittent nature, as is seen in foraminal narrowing from
osteophytes. Typically, the inferior nerve root is affected (e.g. C5-6 disc abnormalities
affect the C6 nerve root). C5-6 and C6-7 are the most commonly affected segments.
A C5 radiculopathy typically presents with pain in the shoulder and the upper part of the
lateral arm. Paresthesias are often seen in the more distal part of the affected dermatome.
Deltoid weakness is seen commonly with a C5 radiculopathy. Biceps or brachioradialis
weakness can be seen with a C6 radiculopathy along with the appropriate hyporeflexia.
Paresthesias and frank sensory loss are more distal, and can extend into the hand. Root
compression at C7 produces triceps weakness and a decreased triceps reflex. Pain
extending into the distal forearm or hand is common. Sensory loss is commonly seen in
the hand.
Lumbar
Sciatica is a classic syndrome of lower lumbar nerve root compression. Low back pain,
that may or may not have been associated with some sort of trauma, is commonly
antecedent to the onset of leg pain by days to a few weeks. Pain tends to be more
proximal, and in a slightly different distribution than sensory changes. Motor weakness is
also seen, but can be missed if dynamic testing is not done. All patients should be asked

77
to stand on their toes and heels, as confrontational testing will miss subtle motor deficits
in the lower extremities. As in the cervical spine, the pathologic level usually affects the
caudal nerve root (e.g. L5-Sl disc produces an S1 radiculopathy). L5-S1 and L4-5 are
overwhelmingly the most common levels affected. The upper lumbar spine is affected
less frequently.
The classic S1 radiculopathy results in pain down the back of the leg and into the heel or
foot. Sensory loss is usually over the lateral aspect of the foot. Plantar-flexion weakness
is seen, but can be subtle. A loss of the Achilles reflex is also fairly specific to S1. The L5
radiculopathy produces similar pain, but the sensory symptoms tend to be over the
dorsum of the foot. Weakness in dorsiflexion of the foot (or more specifically extensor
hallicus longus) is the motor finding associated with L5. There is not a reliably
reproducible reflex associated with L5.
Cauda Equina Syndrome
The cauda equina syndrome is important to recognize, as prompt surgical attention may
be necessary in cases of acute pathology. Patients typically present with low back pain
and diffuse lower extremity complaints. Minimal or absent leg pain is seen, in contrast to
the predominance of extremity pain seen in the radicular syndromes. Bowel, and, more
commonly, bladder dysfunction, are also seen. In many patients this goes unrecognized
until bladder distention leads to overflow incontinence. Because of the vague nature of
the complaints, and the common lack of severe pain associated with a large disc
herniation producing a cauda syndrome, delay in diagnosis is not uncommon. Patients
with back pain and complaints of lower extremity weakness should be carefully
examined to rule out a cauda equina syndrome. Weakness can be diffuse, and vary from
subtle to paraplegia. Sensory findings are variable, but often found in the perineal area.
Checking a post void residual can give a quick initial assessment of bladder function, and
should be done prior to placing a foley. Reflex changes are variable, but in general reveal
diffuse hyporeflexia. Patients in whom the diagnosis is suspected should undergo urgent
imaging with MRI or a myelogram.
Recovery from a severe cauda equina syndrome, even with prompt surgical management,
can be very slow and incomplete. Bladder function is often the slowest symptom to
improve. This is another reason to keep a high index of suspicion for this uncommon
entity in patients presenting with back pain and complaints of lower extremity weakness.
Myelopathy
Myelopathy is the clinical presentation of pathology affecting spinal cord function. The
differential diagnosis for causes of myelopathy is large and includes trauma, metabolic,
degenerative, inflammatory, toxic, infectious, and neoplastic etiologies. Degenerative
conditions of the spine may produce the symptoms of myelopathy. In many instances the
onset of the myelopathy is insidious, and symptoms and signs subtle. Longstanding
myelopathy, unfortunately, is rarely reversible. Early identification of patients with
progression of myelopathy is essential to prevent permanent loss of neurologic function.
Therefore, in patients who present with neck or thoracic spine pain, the history and
physical exam should be tailored to exclude myelopathy.
The most general signs of myelopathy are those of upper motor neuron dysfunction.
Subtle symptoms include difficulty with fine motor control of the hands and fingers, gait
problems and instability, and numbness. Hyperreflexia, increased tone, and weakness are
the hallmarks of the clinical exam. Abnormal plantar response and Hoffman’s sign are

78
frequent abnormal reflexes seen in patients with myelopathy. Urinary dysfunction, such
has hesitancy, frequency, and incontinence, is also seen, but tends not to be severe.
Chronic
Progression of the myelopathy can be very slow and gradual, or stepwise. It is not
uncommon for the onset to be so insidious that patients are quite disabled before they
seek medical evaluation for their symptoms. Careful history and examination can direct
the level of suspicion. In general, symptoms that affect the hands and upper extremities
should prompt cervical evaluation, while isolated lower extremity symptoms and a trunk
sensory level are more suspicious for a thoracic lesion. Often in chronic myelopathy the
distinction can be difficult. In patients with chronic myelopathy an MRI is the study of
choice to evaluate the spinal canal. In patients who are unable to have an MRI,
myelography and CT myelography is adequate and gives good information about the
spinal canal.
Acute
Degenerative disease may lead to acute onset of myelopathy. Acute disc herniation can
be seen in the setting of trauma, but also without significant injury. Patients who present
with the acute onset of myelopathic symptoms deserve urgent evaluation with MRI or
myelography. If pathology such as an acutely ruptured disc causing spinal cord
compression is found, surgical evaluation should be sought. Unfortunately, patients with
complete spinal cord injuries only infrequently make full recoveries.
Acute worsening of cervical myelopathy in the setting of cervical stenosis can be seen in
the face of fairly minor trauma. In patients with acute myelopathy without obvious
fracture, but significant degenerative disease, cervical stenosis should be suspected.
Specific Conditions
The Herniated Disc
The central portion of the intervertebral disc is the nucleus pulposus. Under certain
pathologic conditions it may rupture through the annulus fibrosis and into spaces
occupied by neurologic structures. Central, large disc rupture causing compression of the
spinal cord or cauda equina may be seen. It is, however, much more common to find a
posterior-lateral rupture producing nerve root compression and radiculopathy.
Mechanisms producing radicular pain are poorly understood. Direct compressive effects
certainly play a role. The dorsal root ganglion appears especially sensitive to compressive
effects. Recent animal models have suggested a role for biochemical factors leading to
inflammation. Increasingly, experimental evidence suggests that the mechanisms leading
to pain generation are more complex than once thought.
Lumbar
Lumbar radiculopathy is commonly known as sciatica. The classic presentation is in
younger patients who will present with a history of back pain followed in a few days to
weeks by intense leg discomfort, paresthesias, and radicular weakness as described in the
previous section.
Soft disc herniations in the lumbar spine leading to radicular complaints are seen most
often in the 3rd through 6th decade of life. Estimates of prevalence vary widely in the
literature, from as low as 2% to high as 40%.
Non-surgical therapies for symptoms due to lumbar disc herniation are plentiful. The
natural history of radiculopathy is one of improvement in many individuals. A variety of
therapies are successful in helping patients get through very painful periods. Oral or

79
epidural steroids can be quite successful in managing lumbar radiculopathy, although the
results are often temporary. Physical therapy, chiropractic manipulation, and a host of
other devices and regimens are used and promoted. Definitive evidence on the superiority
of any particular approach to non-surgical therapy is lacking. All patients without severe
neurologic deficit should undergo a trial of non-surgical therapy. The duration of non-
surgical therapy is not set, and is often driven by the patient’s ability to continue to
tolerate their symptoms. Frequently 4 weeks, and, preferably 2-3 months of non-surgical
treatment are recommended., It is, however, common for patients with severe pain or
neurologic deficit to be operated upon more quickly.
The lumbar laminectomy for discectomy is one of the most widely performed spinal
procedures. Despite this, indications continue to be debated. Except for patients with a
cauda equina syndrome, non-operative therapy is always an option. There is prospective
data from Weber that would suggest that patients that undergo discectomy improve more
quickly over the short term. This benefit appears to dissipate by 4 years. With those
disclaimers, most spine surgeons would agree that reasonable operative indications would
include 1) large midline disc herniation with resulting cauda equina syndrome; 2) nerve
root compression with pain and significant motor and sensory deficit; 3) nerve root
compression with or without neurologic deficit and incapacitating pain that fails to
improve with non-surgical measures; 4) recurrence of incapacitating episodes of LBP and
sciatica that prevent the patient from leading a normal life.
In patients with clinical radiculopathy and concordant imaging findings a successful
surgical outcome can be expected in 80-95% of patients. Recurrence rates are reported at
2-12%. The incidence of serious complications is very low (<2%).
Cervical
Cervical radiculopathy from nerve root compression can be caused by a herniated disc or
from foraminal narrowing from osteophytes. The root compression syndromes produced
by these conditions have been described above. Neck pain associated with degenerative
disc disease and osteophytes will improve in the majority of people without invasive
treatment; although there is certainly a group that will go on to have chronic symptoms.
The natural history of cervical radiculopathy is not as well characterized as that of
cervical myelopathy from degenerative disease. Radiculopathy will improve with time in
many patients. However, it is impossible to define strict rules on the length of non-
surgical therapy to undertake before surgery should be considered. Cervical radiculitis
from a soft disc herniation may be less likely to improve spontaneously as that due to
osteophytes. Non-surgical therapy can include oral or epidural steroids, cervical traction,
physical therapy, bracing, and many others.
In carefully selected patients with radicular symptoms and evidence of nerve root
compression on their imaging studies, more than 90% can expect a favorable outcome
with careful surgical management. Serious complications are rare (<1%).
Spinal Stenosis
Spinal stenosis is the narrowing of the cross-sectional diameter of the spinal canal to such
an extent that neurologic symptoms or signs are produced. The syndromes produced by
lumbar and cervical stenosis are quite distinct and will be discussed separately.
Lumbar
Lumbar stenosis classically produces neurogenic claudication. Neurogenic claudication is
leg pain produced by walking or standing that is typically relieved by a change of

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position such as squatting, leaning over or sitting down. Leg pain can be in a variety of
distributions, and becomes quite debilitating. Patients often report associated
paresthesias. Neurologic examination may be normal at rest, though sensory deficits and
hyporeflexia are sometimes seen. When motor weakness is found it can be associated
with wasting, as stenosis is usually a slowing progressive disease. Approximately 2/3 of
patients with symptomatic spinal stenosis will present with some variety of the classic
picture of neurogenic claudication.
Acquired spinal stenosis is caused by advanced degenerative disease of the disc, facets
and ligaments. The hypertrophy of the facets and associated ligaments, such as the
ligamentum flavum, combine with bulging discs to produce both central and lateral
narrowing. Most patients with acquired lumbar stenosis are in their 6th to 7th decade or
beyond.
Surgical treatment for lumbar stenosis involves decompressive laminectomy and may
require medial facetectomies for lateral recess stenosis and foraminal stenosis. More
recently, surgeons have been exploring the role of lumbar fusion in the treatment of
spinal stenosis in the older population. The role of fusion and instrumented fusion in this
setting is yet to be fully determined. The reader is referred to the suggested readings for
more on this topic.
Early improvement after surgery for lumbar spinal stenosis is the rule (>90%) in patients
with a postural component to their pain. However, late progression of symptoms is not
uncommon. A large review by Turner et al. suggests that good results are maintained in
approximately 64% of patients over time.
Cervical
Cervical spondylotic myelopathy (CSM) is the clinical entity produced by cervical
stenosis. CSM usually progresses slowly, in a stepwise fashion. This myelopathy can be
quite subtle in the early stages, and some patients will have significant disability before
seeking appropriate medical care. The most common presenting complaints include neck
pain, gait difficulties, and hand numbness and clumsiness. Loss of bowel and bladder
control is uncommon early in CSM. Occasionally patients will present with acute and
profound spinal cord injury after mild trauma (usually a hyperextension injury). More
common is a stepwise decline in spinal cord function.
The typical patient with CSM is older than 50 and male. Men are seen nearly twice as
often as women. Myelopathic findings dominate the physical examination of patients
with CSM. Increased reflexes in both the upper and lower extremities with lower
extremity spasticity are common. Pathologic reflexes such as Babinski and Hoffman are
also often positive. Lhermitte’s sign (electric, shock-like pain radiating down the spine on
neck flexion) is classically described, but occurs in a small minority of patients.
Complicating the clinical picture in CSM is the lower motor neuron findings that can be
seen secondary to nerve root compression. Wasting, fasciculations, and hypoactive
reflexes can be seen in the upper extremities due to nerve root compression.
The differential diagnosis of CSM includes multiple sclerosis, syringomyelia, spinal cord
tumor, subacute combined degeneration, and normal pressure hydrocephalus. Special
care should be taken in patients with both upper and lower motor neuron signs, as
amyotrophic lateral sclerosis and CSM can be difficult to distinguish.
Surgical decompression of the cervical spinal cord will be recommended by most
neurosurgeons in the setting of any signs of myelopathy and significant cervical canal

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stenosis. Deficits acquired by patients with CSM are rarely completely corrected by
surgery, so most surgeons will tend to offer decompression as early as possible. In
patients with significant cervical stenosis without signs or symptoms of myelopathy
operative indications are less clear. The role of fusion in the treatment of CSM is debated,
and is beyond the scope of this chapter.
Surgical results in the large series available suggest that in 75-90% of cases the
myelopathy can be stabilized or improved. The incidence of worsening of myelopathy
with surgery is low (<1%). Other complications are approach- related and the reader is
referred to the suggested readings.
Diagnosis and Management of Peripheral Nerve Injury and Entrapment
This review is intended to present a set of general principles which can be applied
clinically to both evaluate and treat a broad spectrum of peripheral nerve problems which
include traumatic injuries and associated entrapment neuropathies.
Peripheral nerve injuries
Clinical evaluation

Peripheral nerve injury or disease can cause symptoms of pain, dysesthesias, and either
partial or complete loss of sensory and motor function. A thorough clinical history,
physical examination, electrodiagnostic evaluation, and relevant radiographic studies
should be performed to distinguish a peripheral nerve problem from one involving the
spinal cord or brain, bone, or soft tissues. In addition, early neurosurgical consultation
should be obtained.
The strength of individual muscles or muscle groups is graded. A sensory exam is
performed which includes testing for light touch, pinprick, two-point discrimination,
vibration, and proprioception accordingly. It is helpful to test sensation in the
autonomous zones of a nerve where there is minimal overlap from adjacent nerves. The
presence of Tinel's sign is useful to localize a nerve injury. The Tinel's sign refers to
paresthesias elicited by tapping along the course of a nerve. Progressive distal
advancement of a Tinel's sign over time can be useful clinically to follow the course of
regenerating sensory axons. However, the presence of a Tinel’s sign does not guarantee
motor recovery. Return of sweating in an autonomous zone signifies sympathetic nerve
fiber regeneration. Reflex changes are also sensitive and early indicators of nerve
damage.
Both electromyography (EMG) and nerve conduction studies (NCS) are useful to
distinguish an upper from lower motor neuron disorder as well as diagnose a primary
muscle disease. For EMG studies, a needle electrode is placed through the skin into a
specific muscle and the activity at rest and electrical response to graded muscle
contractions are determined. The nerve conduction study involves stimulation and
recording along the course of peripheral nerves. This allows one to measure the velocity
and amplitude of the propagating nerve action potential.
An understanding of the functional anatomy of the peripheral nervous system can often
permit the clinician to localize nerve injuries and lesions with a high degree of accuracy:
The brachial plexus typically originates from the fifth through eighth cervical spinal
nerve roots as well as the first thoracic root and innervates all of the muscles of the upper
extremity. These nerve roots join to form trunks, which further subdivide into divisions,
then cords, then, more distally, individual peripheral nerve branches. Severe trauma

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transmitted to the most proximal portions of the brachial plexus may produce a
preganglionic injury with avulsion of spinal nerve roots from the spinal cord. It is
important to determine whether avulsion of the ventral or dorsal roots has occurred, since
direct repair of a peripheral nerve whose contributing spinal roots have been disconnected
from the spinal cord will not restore function. A Horner's syndrome, characterized by
ptosis, miosis, and anhidrosis indicates avulsion of the ipsilateral proximal C8 and/or T1
spinal nerve roots. Other physical signs of proximal nerve root avulsion are elevation of
the ipsilateral hemidiaphragm (phrenic nerve), scapular winging (long thoracic nerve),
and weakness of the rhomboid muscles (dorsal scapular nerve). All of these nerves
originate proximally along spinal nerves.
The lumbo-sacral plexus arises from the first lumbar through the fourth sacral spinal
nerves. The femoral and obturator nerves arise from the anterior divisions of L2-4. The
sciatic nerve is the largest nerve in the body and arises from the L4-S4 spinal nerves. This
nerve passes through the sciatic notch and travels down the back of the leg where it
branches into peroneal and tibial nerves usually just above the popliteal fossa.
Imaging of peripheral nerve lesions
Imaging techniques such as X-rays, CT, and, most recently, MRI can be valuable
diagnostic tools in evaluating peripheral nerve lesions. Cervical spine fractures are
frequently associated with brachial plexus injuries, as well as injuries of the proximal
spinal nerves and roots. Chest radiographs may show unilateral elevation of the
diaphragm as a signature of phrenic nerve paralysis (C3-5) from injury to the proximal
upper cervical spinal nerves and roots. Mid-humeral fractures are associated with radial
nerve injuries while midforearm fractures of the ulna or radius are associated with
median or ulnar nerve injuries, respectively. Hip and proximal femur fractures are
associated with sciatic nerve injuries while more distal femur fractures are associated
with peroneal or tibial nerve injuries.
Myelography in conjunction with a CT scan are useful to visualize meningeal diverticula
and abnormalities of the spinal nerve roots, findings of which also indicate a spinal nerve
root avulsion injury.
CT is able to delineate soft tissue mass lesions such as tumors. MRI has proven to be
much more effective in resolving the fine anatomical detail of soft tissues. Using
conventional and enhanced MRI techniques, it has been possible to visualize both normal
and abnormal peripheral nerve structures. New techniques such as MRI "neurography"
make it possible to image and reconstruct the complex peripheral nerve anatomy as well
as pinpoint regions of pathology. MRI can also be used to image signal changes in
denervated muscle.
Grading of peripheral nerve injury
The severity or grade of a peripheral nerve injury is determined by the magnitude and
duration of the applied forces of injury. Seddon defined 3 grades of nerve injury
(neurapraxia, axonotmesis, and neurotmesis ) based on the extent of injury to the three
structural components of the peripheral nerve described above.
Neurapraxia, the mildest grade of nerve injury, is characterized by a reduction or
complete blockage of conduction across a segment of nerve. Axonal continuity is
maintained and nerve conduction is preserved both proximal and distal to the lesion but
not across the lesion. Neurapraxia can result from direct mechanical compression,
ischemia secondary to vascular compromise, metabolic derangements, or diseases or

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toxins causing demyelination of the nerve. Conduction is restored once either the
metabolic derangement is corrected or remyelination occurs. Neurapraxic injuries are
usually reversible and a full recovery can occur within days to weeks.
Axonotmesis represents a more severe grade of nerve injury and is characterized by
interruption of the axons with preservation of the surrounding connective tissue
"highway" which can support axonal regeneration. Distal Wallerian degeneration (axon
and myelin degenerate distal to site of injury) of the axons occurs over a several day
period after which direct electrical stimulation of the disconnected distal nerve stump will
not give rise to a nerve conduction or muscle response. Recovery can occur through
axonal regeneration due to the preservation of the connective tissue "highway" which
consists of Schwann cells and their basal lamina. The Schwann cells proliferate and form
longitudinal conduits (i.e. the bands of Bungner) through which axons regenerate.
Axonotmetic injuries usually recover over a period of months. The timing and degree of
recovery depends on several factors which include the extent of retrograde axonal loss, as
well as the time to regenerate and reinnervate target muscles and/or sensory end organs.
As a general rule, peripheral nerve fibers regenerate at a rate of approximately 1 mm per
day or 1 inch per month. Therefore, more proximal injuries require longer time intervals
for regenerating axons to reinnervate their targets.
Neurotmesis is the severest grade of peripheral nerve injury. Neurotmetic injuries are
characterized by disruption of the axon, myelin, and connective tissue "highway"
components of the nerve. Therefore, recovery through regeneration cannot occur. This
grade of injury encompasses nerve lesions where external continuity of the nerve is
preserved but intraneural fibrosis occurs and blocks axonal regeneration. Neurotmetic
injuries also include nerves whose continuity has been completely interrupted. Since the
necessary "highways" for axonal regeneration are absent, surgery is required to remove
any intervening roadblocks in the form of scar tissue as well as to re-establish continuity
of the nerve.
On the basis of clinical symptoms and physical findings alone, it is often difficult to
differentiate neurapraxic, axonotmetic, and neurotmetic grades of nerve injury, especially
in the acute setting. Nerve conduction studies, both sensory and motor, are useful during
and after the first week following an injury to distinguish neurapraxic from axonotmetic
and neurotmetic grades.
Treatment Strategies:
Trauma is the most frequent cause of peripheral nerve lesions. Nerve injuries are caused
by traction, compression, sharp laceration, and missile injury (gun shot wounds). Traction
injury is often associated with a fracture or dislocation.. An understanding of the
mechanism of injury is extremely helpful in determining the severity of the lesion and to
guide clinical management.
Traumatic peripheral nerve injuries can be classified into open and closed injuries.
Decision making for open injuries is relatively straightforward. Immediate repair of acute
sharp lacerating injuries (i.e. glass or knives) should be undertaken with the goal of
performing a primary end to end suture repair. However, not all transecting injuries lead
themselves to a primary repair. It the ends are ragged or contused, a delayed repair is
preferable to demarcate normal from abnormal neural tissue.
The decision making process in treating closed traumatic peripheral nerve injuries is
more complex. The majority of closed traumatic injuries are due to stretch and/or

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compressive forces. An associated expanding hematoma producing a compartment
syndrome may require emergent surgery to avoid irreversible nerve injury. Because
nerves are often contained in a neurovascular bundle, there is potential for combined
vascular and neural trauma A delayed onset of a neural deficit due to a traumatic
pseudoaneurysm may also require urgent attention. An angiogram is necessary when
damage to vascular structures is suspected clinically. In the majority of closed traumatic
injuries, however, nerves are not actually transected. Instead, a "lesion in continuity"
representing the damaged segment of nerve may be produced which results in either a
neurapraxic, axonotmetic, neurotmetic, or combination of these grades of injury.
In the case of compression or stretching, it if often not possible to immediately determine
the grade of the injury. A partial nerve injury associated with muscle denervation usually
indicates an axonotmetic grade of injury. Patients with such injuries should be followed
with serial clinical and electrodiagnostic examinations to document recovery and confirm
the diagnosis. These patients do not require immediate surgical intervention. An
enlarging hematoma can convert a partial nerve injury into a complete injury. Complete
nerve injuries produce severe muscle denervation and may represent either an
axonotmetic or neurotmetic grade of injury. It is critical to distinguish between these two
grades of injury over time, since the latter requires a surgical repair for recovery to occur.
Patients are therefore followed closely over a several month period looking for clinical
and electrodiagnostic evidence of nerve regeneration and muscle reinnervation.
Muscles should be reinnervated within two years following a traumatic nerve injury if
recovery of useful motor function is to occur. Beyond this point, denervated muscles
undergo irreversible atrophy and replacement with fat. Therefore, it is necessary to time a
surgical exploration so that a successful nerve repair results in muscle reinnervation
within two years of the injury. A useful rule of thumb is to follow a patient for 3 to 4
months to allow any element of neurapraxia to resolve as well as permit axonal
regeneration to occur beyond the point of injury. If there is no clinical or
electrodiagnostic evidence of muscle reinnervation, then a surgical exploration using
intraoperative electrophysiological monitoring should be performed.
Another approach in the management of these traumatic peripheral nerve injuries is to
operate "early" (i.e. as soon as medically feasible). The rationale for this approach is the
following: 1) less scarring and thereby easier dissection of peripheral nerve elements; and
2) intraoperative evaluation of anatomical and electrophysiological continuity. However,
it remains controversial whether an earlier surgical repair leads to a better recovery of
peripheral nerve function.
Entrapment neuropathies
Peripheral nerve entrapment describes the mechanical irritation by which a specific
peripheral nerve becomes locally injured in a vulnerable anatomic site. Peripheral nerve
entrapments produce focal disturbances of nerve function. Nerve entrapment may occur
at any site as a result of non-specific local lesions including fracture callus, hematomas,
and benign or malignant tumors. There are several anatomical sites where peripheral
nerves run in relatively confined spaces and are therefore at increased risk of
compression. The differential diagnosis of entrapment neuropathies includes any disease
process that damages nerves in a focal manner: i.e. degenerative, hereditary, vascular,
inflammatory, and metabolic. Predisposing factors include repetitive activities involving
the affected extremity, tenosynovitis, rheumatoid arthritis, acromegaly, alcoholism,

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amyloidosis, mucopolysaccharidosis, gout, sarcoid, vitamin B6 deficiency, diabetes,
trauma, and conditions altering fluid balance including pregnancy, oral contraceptives,
and hypothyroid myxedema.
Carpal tunnel syndrome
Median nerve compression beneath the flexor retinaculum of the wrist is the most
common entrapment neuropathy. Women>men 2:1
Symptoms;Intermittent numbness and paresthesias along flexor aspects of thumb, index,
and middle fingers, as well as radial side of 4th finger with or without pain, pain may
radiate to the forearm and upper arm, symptoms are worse with repetitive use of the
hand, pain awakens patients from sleep
Examination; Phalen’s maneuver (flexion of wrist with elbow extended for 60 seconds
reproducing symptoms, reverse Phalen’s maneuver (extension of wrist for 60 seconds),
tinel’s sign (localized pain or paresthesia in the cutaneous distribution of the nerve when
it is percussed), sensory loss in median nerve distribution (altered light touch and later
two-point discrimination), thenar muscle wasting (LOAF muscles: lumbricals I,II,
opponens pollicis, abductor pollicis brevis, flexor pollicis brevis)
Diagnostic studies; nerve conduction studies show localized slowing of nerve
conduction velocity or decreased sensory amplitude in the sensory fibers across the wrist,
signs of muscle denervation of thenar musculature on EMG (electromyogram) with
advanced disease
Differential Diagnosis; C6 radiculopathy, proximal median nerve compression, anterior
interosseus syndrome, lateral cord of brachial plexus compression, raynaud’s disease /
vascular, generalized peripheral neuropathy, amyotrophic lateral sclerosis
Treatment
Nonsurgical; Avoid precipitating activity, volar wrist splint in neutral position,short
course of nonsteroidals or prednisone,local steroid injection into carpal tunnel, diuretic if
premenstrual, recommended for patients with mild, intermittent, or acute symptoms.
Surgical; Carpal Tunnel release. Indicated for thenar muscle weakness or atrophy,
denervation by EMG (axonotmesis), failure of nonsurgical management
Ulnar Nerve Entrapment
Ulnar nerve entrapment in the region of the elbow is the second most frequently seen
compression neuropathy. As the ulnar nerve descends down the arm it becomes
superficial behind the medial epicondyle at the elbow. At this point it travels between the
heads of flexor carpi ulnaris (cubital tunnel) and finally passes through the ulnar tunnel
(Guyon’s canal) to enter the hand. The anatomic cubital tunnel is a fibroosseous ring
formed by the medial epicondyle and the proximal part of the ulna. The ulnar nerve is
vulnerable to compromise from compression, scar fixation, or traction, as it winds around
the medial epicondyle. Patients subjected to immobilization (e.g. anesthesia, coma,
restrained positions) are at risk for prolonged pressure on the ulnar nerve.
Symptoms; Pain at the elbow, paresthesias in ulnar side of 4th and 5th digits (palm and
dorsum),exacerbated with repetitive flexion
Diagnosis; Weakness of pinching, grip, 4th and 5th flexors, positive Froment’s sign
(Inability to adduct the thumb against the index finger without flexing the interphalangeal
joint), weakness of third palmar interosseous with abduction of 5th digit (Wartenberg’s
sign), clawing posture of little and ring fingers (benediction posture), point tenderness

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(Tinel’s sign) above elbow (ligament of Struthers), at elbow (trauma), or below elbow
(cubital tunnel), with radiation into the 4th and 5th fingers.
Electrodiagnostics show motor nerve conduction slowing across the elbow, reduced
sensory action potential, and denervation in ulnar innervated muscles (intrinsic hand
muscles)
Differential Diagnosis; ulnar neuropathy at Guyon’s canal in the hand,C8 radiculopathy,
thoracic outlet syndrome (medial cord of brachial plexus C8-T1), Raynaud’s disease
Treatment;
Nonsurgical; Avoid repetitive flexion and pressure on the nerve, splint elbow in
extension, elbow pad.
Surgical; Ulnar nerve decompression and/or anterior transposition (subcutaneous,
intramuscular, or submuscular) if progressive deficits or objective weakness
Thoracic outlet syndrome
The brachial neurovascular bundle goes through the thoracic outlet to enter the arm.
Thoracic outlet syndrome is caused by bony, fascial, and muscular structures that
interfere with the neurovascular bundle. A fibrous band within the scalenius anterior
muscle, a cervical rib, or its remnant may result in angulation or compression of the
lower trunk of the brachial plexus or C8/T1 roots and subclavian vessels.
Diagnosis; Paresthesias in forearm and hand commonly precede the development of pain,
atrophy of intrinsic hand muscles, pain or paresthesias when arms held overhead, sensory
loss in territories of ulnar and medial cutaneous nerves, Adson’s maneuver (obliteration
of the pulse with a full breath and head in extension or turned to side)
Treatment;
Nonsurgical; Corset to prevent elevation of the arms or hands, mild Symptoms may
respond to stretching physiotherapy
Surgical; Exploration for refractory symptoms
Meralgia Paresthetica
Entrapment of the lateral femoral cutaneous nerve is referred to as meralgia paresthetica
(meros=thigh; algo=pain). The lateral femoral cutaneous nerve is a branch of the L2 and
L3 nerve roots and is purely sensory. It exits the pelvis to enter the thigh at the upper
lateral end of the inguinal ligament. The most frequent location of entrapment is medial
to its origin on the anterior iliac spine.
Symptoms; Numbness, burning, or tingling of lateral thigh, positive Tinel’s at the level
of the inguinal ligament, worse standing or extending the leg, better sitting, associated
with obesity and/or pregnancy
Treatment;
Nonsurgical; Weight loss, remove constricting binders, corsets, tight belts, tight jeans
Surgical; Steroid/local anesthetic test infiltration around the nerve at the inguinal
ligament, lateral femoral cutaneous nerve surgical decompression (high recurrence rate)
or proximal transection of nerve

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