NEUROLOGY

Dr. Ma.Victoria Valerio- Banatao

NEUROLOGIC EVALUATION

A. HISTORY
 most impt component of the eval of a child with a neuro. problem
 child’s dev’tal assessment is the most impt component
 family hx is extremely impt

B. NEURO EXAM
1. Head
 size & shape shld be documented
 2 fontanels at birth
 posterior fontanel is usually closed & nonpalpable after the 1st 6-8 wks of life
 ant fontanel may normally close as early as 9 mos.; ave. time of closure is 18 mos.
 fontanel is slightly depressed & pulsatile, best evaluated when infant is held upright while asleep or feeding
 ave. rate of head growth in PT: 0.5 cm in the 1st 2 wk, 0.75 cm during the 3rdwk & 1 cm in the 4th wk &
thereafter until the 40th wk of dev’t
 HC of term infant: at birth – 34-35 cm
6mos – 44 cm
1y/o – 47 cm
2. Cranial Nerves (review your cranial nerves)
3. Motor exam
 includes an assessment of the integrity of the MSK system & search for abn mov’ts that may indicate a
disorder of PNS or the CNS
 components include testing of strength, muscle bulk, tone, posture, locomotion & motility, DTRs &
presence of primitive reflexes

MUSCLE POWER GRADING:

0 = no contraction
1 = flicker or trace of contraction
2 = active mov’t,gravity eliminated
3 = active mov’t against gravity
4 = active mov’t against gravity & resistance
5 = normal power

ABNORMALITIES OF TONE:
1. Spasticity
 initial resistance to passive mov’t ff a sudden release called the clasp-knife phenomenon, most
apparent in the upper extremity flexors & LE extensor muscles
 results from a lesion that involves upper motor neuron tracts, maybe unilat/bilat.
2. Rigidity
 result of a basal ganglia lesion, charac by constant resistance to passive mov’t of both extensor &
flexor muscles
 Children w/ spastic LE drag the legs while crawling (commando style) or walk on tiptoes.
 Opisthotonus – marked spasticity & rigidity; head & heels are bent backward & body bowed forward
 Decerebrate rigidity – marked extension of extremities resulting from dysfx or injury to brainstem
3. Hypotonia
 abnormally diminished tone, most common abnormality of tone in neurologically compromised PT or
FT

ABNORMALITIES IN MOTILITY & LOCOMOTION:

1. Ataxia – incoordination of mov’t or disturbance of balance

Abnormalities assoc w/ cerebellar lesions:

a. Dysmetria
b. Rebound
c. disdiadochokinesia

2. Chorea – involuntary mov’ts of the major joints, trunk & the face that are rapid & jerky.
 hand grip contracts & relaxes (milkmaid sign), speech is explosive & inarticulate

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 3. Athetosis - slow, writhing mov’t that is assoc often with abnormalities of muscle tone, most prominent in the
distal extremities & is enhanced by voluntary activity or emotional upset.

4. Dystonia = involuntary, slow, twisting mov’t that primarily involves the prox muscles of the extremities, trunk &
neck

5. Motor tics – sudden brief unsustained mov’t, suppressible & preceded by a warning sensation
 eye blinking, grimacing, brief rapid mov’t of head & shoulders

DTRs
 graded from 0 (absent) to 4 (markedly hyperactive)
 with 2 being normal
 absent or decreased in primary dso of muscle, nerve & myoneural junction & abn of cerebellum

Babinski reflex
 indicates an UMN lesion; characterized by extension of great toe & fanning of remaining toes

Primitive reflexes
 appear & disappear in sequence during specific periods of dev’t.
 their absence or persistence beyond a given time frame signifies dysfx of CNS

4. Sensory exam
5. Gait – a spastic gait is charac by stiffness & by stepping like a tin soldier
 cerebellar ataxia produces a broad based unsteady gait
 a waddling gait results from weakness of the prox hip girdle
 scoliosis may cause an abn gait & result from disorders of muscle & SC

SOFT NEUROLOGIC SIGNS :

 defined as a particular form of deviant performance on a motor or sensory test in the neuro exam that is
abnormal for a particular age
 interpreted cautiously bec they are present in normal children during various stages of neurodevelopment
 variation in expression of these signs , depending on age, sex & maturation of nervous system

C. DIAGNOSTIC PROCEDURES
1. Lumbar puncture – lat recumbent position
 ideal interspace is L3-L4 or L4-L5
 Contraindications:
a. a. elevated ICP 2’ suspected mass lesion of brain or SC
b. s/sx of pending cerebral herniation
c. critical illness
d. skin infection at site of LP
e. thrombocytopenia
2. Subdural tap – to establish a dx of subdural effusion or hematoma
 approached at lat border of ant fontanel or along the upper margin of coronal suture at least 2-3 cm fr
the midline

3. Ventricular tap – used to remove CSF in increased ICP assoc with hydrocephalus
4. Neuroradiologic procedures – skull xrays, CT scan, MRI, radionuclide brain scan
5. EEG – provides continuous recording of electrical activity with electrodes placed on the scalp

CONGENITAL ANOMALIES OF THE CNS

A. NEURAL TUBE DEFECTS (DYSRAPHISM)

 failure of the neural tube to close spontaneously bet the 3rd & 4th wk of in utero dev’t
 precise cause is unknown

1. SPINA BIFIDA OCCULTA

 consists of a midline defect of vetebral bodies without protrusion of SC or meninges
 asymptomatic, lack neuro signs
 patches of hair, lipoma, discoloration of skin or dermal sinus in the midline of low back signifies a
malformation

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 2. MENINGOCELE
 formed when meninges herniate through a defect in the post vertebral arches
 SC usually normal
 (+) fluctuant midline mass that transilluminate along the vertebral column
 well covered with skin

3. MYELOMENINGOCELE
 most severe form of dysraphism
 unknown cause
 genetic predisposition
 maternal periconceptional use of folic acid reduces incidence by at least 50%
 folic acid supplementation shld be initiated before conception & continued until 12 th wk AOG
 produces dysfx of many organs
 lesion in low sacral region causes bowel & bladder incontinence with anesthesia in the perineal
area
 lesion in the midlumbar region produces lower motor neuron signs
 increasing neuro deficit as the lesion extends higher into the thoracic region
 Hydrocephalus in assoc w/ type II Chiari defect develops in at least 80% of patients
 Tx – multidisciplinary team approach
 Prognosis – MR 10-15%, most deaths occur before 4 y/o
 70% of survivors have normal intelligence, but learning problems & seizure dso more common
 chronic handicapping condition

4. ENCEPHALOCELE
 2 major forms of disraphism affect the skull, resulting in protrusion of tissue through a bony midline
defect, called cranium bifidum
 cranial meningocele – consists of a CSF-filled meningeal sac only
 cranial encephalocele – contains the sac plus cerebral cortex, cerebellum or portions of
brainstem
 occurs most commonly in the occipital region but in certain parts of the world, frontal or nasofrontal
are more prominent
 infants with cranial encephalocele are at increased risk for hydrocephalus
 (+) small sac w/ pedunculated stalk or large cystlike structure that may exceed size of cranium
 lesion may be completely covered w/skin
 UTZ – most helpful in determining contents of the sac

5. ANENCEPHALY
 presents a distinctive appearance with a large defect of the calvarium, meninges &
scalp assoc with a rudimentary brain
 results fr failure of closure of the rostral neuropore, opening of the anterior neural tube
 primitive brain consists of portions of CT, vessels & neuroglia
 die w/in several days of birth

6. MICROCEPHALY
 HC that measures more than 3 standard deviations below the mean for age & sex
 common among the mentally retarded
 2 Main Grps
 primary microcephaly – group of conditions that usually have no other malformations & follow a
mendelian pattern of inheritance or are assoc w/ a specific genetic syndrome
 secondary microcephaly – results from a large number of noxious agents that may affect a fetus
in utero or an infant during periods of rapid brain growth , particularly the 1 st 2 yrs of life

B. HYDROCEPHALUS
 not a specific dse, represents a diverse grp of conditions that result from impaired circulation & absorption of CSF
or increased production by a choroid plexus papilloma
 normal CSF prodn – 20 ml/hr
 total vol – 50 ml in infants

 Obstructive or noncommunicating hydrocephalus – hydrocephalus resulting from obstruction within the

ventricular system

 Nonobstructive or communicating hydrocephalus – hydrocephalus resulting from obliteration of the

subarachnoid cisterns or malfunction of the arachnoid villi

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  Clinical manifestations:
 accelerated rate of enlargement of head
 wide open, bulging ant fontanel
 dilated scalp veins, broad forehead
 setting-sun eye sign
 (+) Macewen sign – percussion of skull produce a cracked-pot, indicating separation of sutures

C. CRANIOSYNOSTOSIS
 premature closure of cranial sutures
 Classification:
1. Primary – closure of one or more sutures due to abn of skull dev’t
 incidence approximates 1/2000 births
 unknown cause
 genetic syndromes account for 10-20% of cases
2. Secondary – results from failure of brain growth & expansion

 Clinical Manifestations:
 evident at birth, characterized by skull deformity
 palpation of suture reveals prominent bony ridge
 scaphocephaly – premature closure of sagittal suture producing a long & narrow skull; most common form of
craniosynostosis
 frontal plagiocephaly – next most common; unilat flattening of forehead, elevation of ipsilat orbit or
eyebrow; premature fusion of coronal & sphenofrontal suture

SEIZURES IN CHILDHOOD
 Seizure or convulsion – paroxysmal, time limited change in motor activity &/or behavior that results fr
abn electrical activity in the brain.
 occur in approx 10% of children
 less than one third of seizures in children are caused by EPILEPSY, a condition in w/c seizures are triggered
recurrently from w/in the brain
 EPILEPSY – considered when 2 or more unprovoked seizures occur at an interval greater than 24 hrs apart

A. FEBRILE SEIZURES
 most common seizure dso in childhood
 excellent prognosis
 may signify a serious underlying acute infectious dse such as bact meningitis
 age dependent, rare before 9 mos & after 5 y/o
 genetic predisposition
 peak age of onset – 14-18 mo of age
 febrile seizure gene – chromosomes 19p & 8q13-21

 Clin. Manif:
 GTC seizure, lasts for few sec to 10 mins
 followed by brief postictal period of drowsiness
 viral URTI,roseola & acute otitis media are frequent causes
 Complex/complicated febrile seizure: >15 min duration; repeated convulsions occur w/in 24 hr or when focal sz
activity or focal findings are present during postictal period

B. UNPROVOKED SEIZURES

1. First seizure
 LP is of limited value in a child w/ a 1st nonfebrile seizure
 anticonvulsants generally not recommended after a single seizure
 EEG recommended in a child w/ an apparent 1st unprovoked seizure
2. Recurrent seizures
 2 unprovoked seizures greater than 24 hrs apart suggest presence of an epileptic dso w/in the brain
that will lead to future recurrences

C. PARTIAL SEIZURES
 account for 40% 0f cases
 classified as simple or complex
 consciousness is maintained with simple sz, impaired in complex sz

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 1. SIMPLE PARTIAL SEIZURES (SPS)
 motor activity is the most common sx
 asynchronous clonic or tonic mov’t involving the face,neck & extremities
 automatisms do not occur but some may complain of aura (chest discomfort,headache)
 versive seizures consisting of head turning & conjugate eye mov’ts are common
 (+) aura
 10-20 sec
 distinguishing charac: px remain conscious & may verbalize during the seizure
 no postictal phenomenon
 EEG – spikes or sharp waves unilat or bilat or multifocal spike pattern

2. COMPLEX PARTIAL SEIZURES (CPS)

 may begin with a SPS with or w/o aura ff by impaired consciousness
 presence of an aura always indicates a focal onset of the seizure
 (+) automatisms – common feature; occurs in 50-75% of cases; develop after the LOC & may persist
into the postictal phase; not recalled by child
 prolonged & repetitive alimentary automatisms assoc with blank stare or with lack of
responsiveness almost always indicate CPS in an infant
 ave duration – 1-2 min
 tends to be observed during waking hours

3. BENIGN PARTIAL EPILEPSY WITH CENTROTEMPORAL SPIKES (BPEC)

 excellent prognosis
 occurs bet. 2-14 y/o, peak age of onset is 9-10 y/o
 occurs in normal children w/ unremarkable hx & normal neuro exam
 (+) FHx of epilepsy
 occurs during sleep in 75% of px
 clinical features, EEG findings(rolandic foci) & lack of neuropathologic lesion are characteristic &
separate BPEC from CPS
 seizures usually partial; motor signs & somatosensory symptoms are confined to face
 oropharyngeal symptoms : tonic contractions & paresthesias of tongue, unilat numbness of cheek,
guttural noises, dysphagia & excessive salivation
 consciousness may be intact or impaired
 Carbamazepine – preferred drug; continued for at least 2 yr or until 14-16 yrs old, when spontaneous
remission of BPEC usually occurs

D. GENERALIZED SEIZURES

1. ABSENCE SEIZURES (PETIT MAL)

 sudden cessation of motor activity or speech w/ a blank facial expression & flickering of eyelids
 uncommon before 5 y/o
 prevalent in girls
 never assoc with aura, not assoc w/ postictal state
 rarely persist longer than 30 seconds
 countless seizures daily; do not lose body tone but head may fall forward slightly

2. GENERALIZED TONIC-CLONIC SEIZURES (GTC)

 may follow a partial seizure w/ a focal onset
 may be assoc w/ aura
 px suddenly lose consciousness, emit a shrill, piercing cry
 eyes roll back,entire body undergoes tonic contractions, & become cyanotic in assoc w/ apnea
 clonic phase is heralded by rhythmic clonic contractions alternating w/ relaxation of muscle
grps
 (+) loss of sphincter control
 postictally, patients are semicomatose, remain in deep sleep fr 30 mins to 2 hrs
 postictal phase is assoc w/ vomiting & intense bifrontal headache

3. MYOCLONIC EPILEPSIES
 repetitive sz consisting of brief , often symmetric muscular contractions w/ loss of body tone & falling
or slumping forward

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 4. INFANTILE SEIZURES
 begin bet ages 4-8 mos
 brief symmetric contractions of the neck, trunk & extremities
 3 types: flexor, extensor & mixed
 a cry may precede or follow an infantile spasm
 may occur during sleep or arousal

 2 GROUPS:

1. Cryptogenic infantile spasms

 has uneventful pregnancy & birth hx; normal dev’tal milestones before onset of seizures
 normal neuro exam; normal CT/MRI scans
 good prognosis

2. Symptomatic infantile spasms

 related directly to several prenatal,perinatal & postnatal factors
 HIE, congenital infections, inborn errors of metabolism,prematurity, CNS infections, head
trauma
 80-90% risk of MR

5. LANDAU-KLEFFNER SYNDROME (LKS)

 rare condition of unknown cause
 more common in boys; mean onset of 5 ½ yr
 characterized by loss of language skills in a previously normal child
 70% have assoc seizure disorder
 Valproic acid – drug of choice

TREATMENT OF EPILEPSY
 1st step – ensure that px has a sz dso
 2nd step – choose an anticonvulsant depending on the classif of seizure, det by the hx & EEG findings

1. Benzodiazepines
 exert its activity by binding to a specific GABA site
 Diazepam & lorazepam IV are used for initial mgt of status epilepticus

2. Carbamazepine
 effective in the mgt of GTC & partial sz
 acts by decreasing the sustained repetitive firing of neurons by blocking Na-dependent channels & by
decreasing depolarization-dependent Ca uptake

3. Phenobarbital
 safe, useful for GTC sz
 25% undergo severe behavioral changes
 affect the cognitive performance of children treated on a long term basis
 acts on the GABA receptor to increase the chloride channel open duration

4. Phenytoin:
 acts by decreasing the sustained repetitive firing of single neurons by blocking Na-dependent channels &
decreasing Ca uptake

5. ACTH:
 preferred drug for infantile spasms

6. Ketogenic Diet
 for mgt of recalcitrant sz
 restricts the quantity of COH & CHON, most calories are provided as fat
 fatty, unpalatable diet
 MOA is unknown but some evidence shows that it exerts an anticonvulsant effect sec to elevated levels of B-
hydroxy-butyrate & acetoacetate resulting from ketosis

7. Surgery
 shld be considered for children w/ intractable sz unresponsive to anticonvulsants

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 8. Vagal Nerve Stimulation
 animal experiments have shown that electrical stimulation of left vagal nerve will interrupt or prevent
seizures

NEONATAL SEIZURES

FIVE SEIZURE TYPES

1. FOCAL SEIZURES
 rhythmic twitching of muscle grps, particularly those of extremities & face

2. MULTIFOCAL CLONIC SEIZURES

 similar to focal clonic sz but many muscle grps are involved

3. TONIC SEIZURES
 rigid posturing of the extremities & trunk, sometimes assoc w/ fixed deviation of the eyes

4. MYOCLONIC SEIZURES
 brief, focal or gen jerks of the extremities or body that tend to involve distal muscle grps

5. SUBTLE SEIZURES
 consists of chewing motions, excessive salivation & alterations in the RR incldg apnea, blinking, nystagmus,
bicycling or pedaling mov’ts

HIE – most common cause of neonatal seizures

STATUS EPILEPTICUS
 continuous convulsion lasting longer than 30 mins or the occurrence of serial convulsions bet w/c there is no
return of consciousness
 medical emergency
 most common cause: febrile sz lasting more than 30 mins , particularly in a child younger than 3 y/o

HEADACHES
 common problem in pediatrics
 may occas indicate a severe underlying dso (brain tumor)
 most impt causes : migraine, increased ICP and psychogenic factors or stress
 less common causes: EOR; strabismus; sinusitis & malocclusion of teeth
 often an associated manifestation of common head & neck infections in children

A. MIGRAINE
 recurrent headache w/ sx free intervals & at least 3 of the ff sx : abd pain, N/V, throbbing headache,unilat
location,assoc aura(visual,sensory,motor),relief ff sleep & a (+) FHx
 most impt & frequent type of headache in pediatrics
 spontaneous prolonged remission after the 10th bday
 cause is unknown
 girls are more likely to develop migraine as adolescents; boys are in slight majority among children younger
than 10 yr

 Cortical Spreading Depression (CSD)

 phenomenon thought to be responsible for the aura in migraine; assoc w/ elevation of CNS H+ & K+ ions
w/ release of glutamate & nitrous oxide
 inherited physiologic response to a variety of stimuli that are responsible for triggering migraine process

CLASSIF OF MIGRAINE

1. COMMON MIGRAINE
 migraine w/o aura
 most prevalent type
 throbbing or pounding, unilat at onset or throughout its duration
 located in the bifrontal or temporal regions
 persists for 1-3 hrs, although pain may last as long as 72 hr
 inhibit daily activity

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  (+) FHX particularly on maternal side is present in 90% of children
 characteristic feature of childhood migraine – intense N/V w/c may be more bothersome than the headache
 vomiting may be assoc w/ abdominal pain & fever
 additional symptoms: extreme paleness, photophobia, light-headedness, phonophobia, osmophobia &
paresthesias of hands & feet

B. CLASSIC MIGRAINE
 migraine w/ aura
 an aura precedes onset of headache
 (+) sensory sx like perioral paresthesias & numbness of hands & feet
 visual auras take the form of blurred vision, scotoma, photopsia, fortification spectra or irregular distortion
of objects
 after the aura, patient develops typical symptoms of migraine

C. MIGRAINE VARIANTS
 these variants include cyclic vomiting, acute confusional states & benign paroxysmal vertigo

a. Cyclic Vomiting : recurrent, sometimes monthly bouts of severe vomiting

 vomiting during attacks occurs at least 5x/hr for at least 1 hr w/ complete resolution of symptoms
between attacks

b. Abdominal Migraine: recurrent disorder characterized by mid-abdominal pain w/ pain-free periods

between each attack
 pain is described as dull, may be moderate to severe; persist from 1-72 hr; usually midline
 child must complain at the time of the abdominal pain of at least 2 of the ff: anorexia, N/V, or
pallor

D. COMPLICATED MIGRAINE
 refers to dev’t of neuro signs during headache that persists after termination of headache

TREATMENT OF MIGRAINE
 avoid initiating stimuli
 most common precipitators : stress, fatigue, anxiety
 AVOID certain foods: nuts, chocolate, cola drinks, hotdog, spicy meats, chinese food, citrus fruits, fried foods,
cheese, yogurt, pocessed meats
 mgt of acute attacks : analgesics & antiemetics
 Acetaminophen (15mg/kg) or Ibuprofen (7.5-10 mg/kg) for mild, infrequent headaches
 Naproxen, ketorolac, codeine, butorphanol & meperidine for severe headache
 Ergotamine prep for older children w/ severe, classic migraine, most efficacious during early stages of migraine
attacks

B. ORGANIC HEADACHES
 headache may be the earliest sx of increased ICP
 headache results from tension or traction of cerebral bld vessels & dura
 occurs in the early hrs in the morning or shortly after the px arises
 headache is diffuse, generalized
 more prominent over the frontal & occipital regions
 insidious onset
 causes: brain tumors, hydrocephalus, meningitis, encephalitis, cerebral abscess, subdural hematoma,
chronic lead poisoning, pseudotumor cerebri

C. TENSION OR STRESS HEADACHE

 uncommon in pedia
 most apparent during the school day
 tend to wax & wane & build in intensity during the day
 not assoc w/ n/v
 px also complain of mood changes, wt loss, anorexia, disturbed sleep, fatigue & withdrawal fr social
activities
 depressed child occasionally presents w/ severe headaches

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NEUROCUTANEOUS SYNDROMES

A. NEUROFIBROMATOSIS (NF)
 von Recklinghausen dse
 AD dso
 consequence of an abnormality of neural crest differentiation & migration
 distinctive features may be present at birth but dev’t of complications is delayed for decades
 café-au-lait spots are the hallmark of NF

 2 FORMS OF NF
1. NF-1 – most prevalent type, susceptible to neurologic complications
2. NF-2 – accounts for 10% of cases, bilateral acoustic neuromas are the most distinctive feature

 Treatment :
 no specific tx
 genetic counseling, early detection of treatable complications

B. TUBEROUS SCLEROSIS (TS)

 AD trait; 1/6000 people
 heterogeneous dse w/ a wide clinical spectrum varying fr severe MR & incapacitating sz to normal
intelligence & lack of sz often w/in the same family
 the younger the px presents w/ s/sx of TS, the greater is the likelihood of MR
 Definite TS complex is diagnosed when at least 2 major or 1 major + 2 minor features are present
 Major features: skin lesions; brain & eye lesions; & tumors in the heart,kidneys or lungs
 Minor features: bone cyst; rectal polyps; dental enamel pits; CNS white matter migrational abnormalities,
gingival fibromas, nonrenal hamartomas, retinal achromic patches, confetti skin lesions & multiple renal
cysts
 tubers – characteristic brain lesion
 greater the number of tubers,the more neurologically impaired
 may present during infancy w/ infantile spasms & hypsarrhythmic EEG pattern
 (+) ash leaf hypopigmented lesions
 (+) shagreen patch is also characteristic of TS; consists of a roughened , raised lesion w/ an orange peel
consistency located in the LS region
 most common neurologic manifestations of TS : seizures; cognitive impairment; behavioral abnormalities
including autism
 approx 50% of children w/ TS have rhabdomyomas of the heart
C. STURGE-WEBER DISEASE
 consists of constellation s/sx including a facial nevus (port-wine stain), sz, hemiparesis, IC calcifications & MR
 occurs in approx 1/50000 LB
 facial nevus is present at birth, tends to be unilateral & always involves the upper face & eyelid

D. VON HIPPEL-LINDAU DISEASE

 AD trait; 1/36000 people
 affects many organs including cerebellum, SC, medulla, retina, kidney, pancreas & epididymis
 major neurologic features: cerebellar hemangioblastomas & retinal angiomata
 renal carcinoma is the most common cause of death

E. LINEAR NEVUS SYNDROME

 charac by a facial nevus & neurodev’tal abnormalities
 nevus is located on the forehead & nose, midline in its distribution
 more than half of px have sz dso & MR

F. PHACE Syndrome
 denotes post fossa malformations, hemangiomas, arterial anomalies, COA or other cardiac defects and eye
abnormalities
 there is female predominance

MOVEMENT DISORDERS

A. ATAXIAS
 inability to make smooth, accurate & coordinated mov’ts
 usually due to dso of cerebellum &/or sensory pathways in the posterior columns of SC

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  may be generalized or primarily affect gait or the hands & arms; they may be acute or chronic
 congenital anomalies of the post fossa assoc w/ ataxia: Dandy-Walker syndrome, Chiari malformation, &
encephalocele
 major infectious causes: cerebellar abscess, acute labyrinthitis, acute cerebellar ataxia
 toxic causes of ataxia: alcohol, thallium (pesticide), anticonvulsants (phenytoin)
 metabolic dso assoc w/ ataxia: abeta-lipoproteinemia, arginosuccinic aciduria & Hartnup dse

 ATAXIA-TELANGIECTASIA:
 AR condition
 most common of the degenerative ataxias
 heralded by ataxia at age 2 y/o & progressing to loss of ambulation by adolescence
 telangiectasia becomes evident by mid- childhood & found on bulbar conjunctiva, over bridge of nose & on
the ears & exposed surfaces of extremities

 FRIEDREICH ATAXIA :
 AR trait
 onset of ataxia occurs before 10 y/o
 ataxia is slowly progressive, involves the LE to a greater degree than the UE
 (+) Romberg test, absent DTRs
 characteristic explosive, dysarthric speech
 (+) nystagmus
 appear apathetic but intelligence is preserved

B. CHOREA, ATHETOSIS, TREMOR

CHOREA
 means “dance” in Greek
 refers to irregular, rapid, uncontrolled, involuntary mov’ts
 result in bizarre mov’ts of the hands & arms as well as abn gait
 Sydenham chorea
 most common acquired chorea of childhood
 sole neurologic manif of RF
 3 major clin manif : chorea, hypotonia & emotional lability
 chorea is symmetric, increased by stress & disappear during sleep
 may persist for several months & as long as 1-2 yr; about 20% experience recurrence w/in 2 yr of initial
episode
 Other causes of chorea
 atypical sz
 drug intoxication (phenytoin)
 hormonally induced sz(oral contraceptives, pregnancy/ chorea gravidarum)
 Lyme disease
 hypoparathyroidism
 hyperthyroidism
 Wilson disease

ATHETOSIS
 distal writhing mov’ts of the extremities
 sometimes combined w/ chorea (choreo-athetosis)
 often seen as part of extrapyramidal CP caused by asphyxia or kernicterus or genetic metabolic disorders

TREMOR
 involuntary mov’t characterized by rhythmic oscillations of a part of the body w/c may be more prominent
during rest or w/ mov’t

 Jitteriness
 rhythmic tremors of equal amplitude around a fixed axis ; most common involuntary mov’t of healthy FT
 most apparent when an infant is crying or being examined (eg. Moro reflex)
 abnormal when infant is awake & alert & when tremor is persists beyond 2nd wk of life
 Organic causes of jitteriness: sepsis, IC hge, hypoglycemia, hypoCa, hypoMg, prenatal exposure to
marijuana, narcotic abstinence syndrome, HIE

Ma. Enrica M.Pattaguan MD 2-A Page 10

  Drugs that cause tremors
 Amphetamines
 Valproic acxid
 Neuroleptics
 Tricyclic antidepressants
 Caffeine
 Theophylline

C. DYSTONIA
 syndrome of sustained muscle contractions, frequently causing twisting & repetitive mov’ts or abn postures
 may be a prominent feature of children w/ extrapyramidal CP
 develop gradually over many years in older children & teenagers after basal ganglia injury

 Major causes of dystonia:

 perinatal asphyxia
 kernicterus
 generalized primary dystonia
 drugs
 Wilson disease
 Hallervorden-Spatz disease
 other genetic mutations
D. TICS
 spasmodic, involuntary, repetitive, stereotyped mov’ts that are nonrhythmic, often exacerbated by
stress & may affect any muscle grp.

1. Transient tic dso

 most common mov’t abnormality of childhood
 more prevalent in boys
 (+) FHx
 consist of eye blinking, or facial mov’t or throat clearing noises
 persists fr wks to less than 1 yr
 does not require drug tx

2. Chronic motor tic dso

 occurs in children & persists throughout adult life
 involve up to 3 muscle grps simultaneously

3. Gilles de la Tourette syndrome

 lifelong condition
 onset bet 2-21 y/o
 AD
 (+) motor tics, vocal tics including throat clearing, sniffling, barking, coprolalia, echolalia, palilalia &
echokinesis

CNS INFECTIONS
 Acute infection of the CNS is the most common cause of fever assoc w/ s/sx of CNS dse in children
 viral infx are more common than bacterial
 common sx : headache, N/V, anorexia, restlessness & irritability
 common signs : fever, photophobia, nuchal rigidity, stupor, coma & focal neurologic deficits

ACUTE BACTERIAL MENINGITIS

 Etiology
0-2 mos grp B & D streptococci
gram (-) enteric bacilli (E.coli, Klebsiella)
L. monocytogenes

2 mo-12y/o S. pneumoniae
N. meningitidis
H. influenzae type b

Ma. Enrica M.Pattaguan MD 2-A Page 11

  less common pathogens
 pseudomonas aeruginosa
 staph aureus
 coagulase negative staph
 salmonella spp

 Epidemiology:
 major risk factor is the lack of immunity to specific pathogens assoc w/ young age
 additional risks : recent colonization w/ pathogenic bacteria; close contact w/ indv having invasive disease;
crowding; poverty; black; male gender
 mode of transmission is probably person to person contact through resp tract secretions

 Clinical Manifestations:
 2 predominant patterns
 more dramatic & less common presentation: sudden onset w/ rapidly progressive manifestations of shock,
purpura, DIC & reduced levels of consciousness often resulting in coma or death w/in 24 hr
 more often presentation, preceded by sev days of fever accompanied by URTI or GI sx ff by nonspecific signs
such as increasing lethargy & irritability
 nonspecific findings: fever; anorexia;poor feeding; headache; URTI symptoms; myalgias; arthralgias;
tachycardia; hypotension; cutaneous signs
 meningeal irritation : nuchal rigidity, back pain, Kernig sign, Brudzinski sign
 (+) seizures, alteration of mental status, photophobia, tache cerebrale
 (+) focal neurologic signs

 Increased ICP is suggested by:

 headache
 bulging fontanel or diastasis of sutures
 oculomotor(anisocoria,ptosis) or abducens nerve paralysis
 hypertension w/ bradycardia
 apnea or hyperventilation
 decorticate or decerebrate posturing
 stupor, coma, signs of herniation

 Diagnosis:
 analysis of CSF
 blood cultures
 CT scan

 Treatment :
 Pen G 300,000 u/kg/day
 Ceftriaxone 100mg/kg/day
 Cefotaxime 200 mg/kg/day
 Chloramphenicol 100 mg/kg/day
 Ampicillin 200mg/kg/day

 if px is immunocompromised & gr (-) bact meningitis is suspected, initial tx are ceftazidime &
aminoglycoside

 duration of antibiotic tx :
 10-14 days for uncomplicated penicillin sensitive S. pneumoniae meningitis
 5-7 days for uncomplicated N.meningitidis meningitis
 7-10 days for uncomplicated H. influenzae type b meningitis

 gram (-) bacillary meningitis shld be treated for 3 wks or for at least 2 wk after CSF sterilization w/c may
occur after 2-10 days of tx

 Complications:
 seizures, increased ICP, CN palsies, stroke, cerebral or cerebellar herniation
 SIADH occurs in majority of px resulting in hypoNa & reduced serum osmolality in 30-50% of infected
children
 Prognosis :
 prognosis is poorest among infants <6 mos & in those w/ > 10 CFU of bacteria/ml in their CSF
 highest MR are observed w/ pneumococcal meningitis

Ma. Enrica M.Pattaguan MD 2-A Page 12

  those w/ sz occuring >4 days into therapy or w/ coma or focal neuro signs on presentation have increased
risk of long term sequelae
 most common neurologic sequelae: hearing loss, MR, sz, delay in acquisition of language, visual impairment
& behavioral problems
 sensorineural hearing loss – most common sequela of bact meningitis

 Prevention:
 vaccination & antibiotic prophylaxis of susceptible at-risk contacts
 chemoprophylaxis is recommended for all close contacts of px w/ meningococcal meningitis regardless of
age or immunization status
 close contacts shld be treated w/ rifampin 10 mg/kg/dose q 12 hr (max dose 600mg) for 2 days

VIRAL MENINGOENCEPHALITIS
 acute inflam process involving the meninges & brain tissue
 CSF is characterized by pleocytosis & absence of microorg on GS & routine bact culture
 self-limited in most instances
 Etiology: enteroviruses (80% of cases), arboviruses, HSV-1, HSV-2, VZV, CMV, EBV
 Epidemiology: infection w/ enterovirus is spread directly from person-person, incubation pd of 4-6 days

 Clinical Manifestations:
 high fever, violent convulsions interspersed w/ bizarre mov’ts & hallucinations alternating w/ brief
periods of clarity ff by complete recovery

 Diagnosis :
 clinical presentation of nonspecific prodrome ff by progressive CNS sx
 CSF exam w/c shows a mild MN predominance
 EEG, neuroimaging studies

 Treatment:
 Acyclovir for HSV
 supportive tx

 Prognosis:
 motor incoordination, convulsive dso, total or partial deafness & behavioral disturbances may
follow viral CNS infections
 Most children recover completely fr viral infections although prognosis depends on the severity of the
critical illness, specific cause & age of the child

EOSINOPHILIC MENINGITIS
 defined as 10 or more eosinophils/mm3 of CSF
 most common cause of eosinophilic pleocytosis is CNS infection w/ helminthic parasites

 Etiology:
 most common cause : rat lungworm Angiostrongylus cantonensis
 non infectious causes: MS, malignancy, hypereosinophilic syndrome or a reaction to medications or a
VP shunt

 Clinical manifestations:
 px become ill 1-3 wk after exposure bec parasites migrate fr the GIT to the CNS
 fever, peripheral eosinophilia, vomiting, abd pain, creeping skin eruptions or pleurisy
 headache, ataxia, CN palsies & paresthesias

 Treatment :
 supportive; infection is self limited
 antihelminthic drugs do not appear to influence outcome
 steroids may decrease duration of headaches in adults

BRAIN ABSCESS
 most common in children 4-8 y/o
 causes : embolization due to CHD (TOF), meningitis, chronic otitis media & mastoiditis, sinusitis, ST infx of face or
scalp, orbital cellulitis, dental infx, penetrating head injuries, immunodef states, VP shunt infection
 most abscesses are single, 30% are multiple

Ma. Enrica M.Pattaguan MD 2-A Page 13

  Etiology:
 S. aureus, strep, anaerobic organisms, gm(-) aerobic bacilli (enteric rods, proteus, pseudomonas)
 sinusitis – anaerobic bacteria

 Clinical Manifestations:
 = nonspec sx – low grade fever, headache, lethargy
 = vomiting, severe headache, sz, papilledema, focal neuro signs (hemiparesis) & coma
 = cerebellar abscess – nystagmus, ipsilat ataxia, dysmetria, vomiting & headache

 Diagnosis :
 aspiration of abscess to establish a bacteriologic dx
 CT & MRI – most reliable methods of demonstrating cerebritis & abscess formation

 Treatment:
 prompt dx & antibiotic regimen based on probable pathogenesis & organism
 unknown cause – 3rd gen cephalosporins & metronidazole
 hx of head trauma or neurosx – nafcillin or vancomycin + 3rd gen ceph + metro
 monotherapy w/ meropenem
 CHD – pen G + metro
 infected VP shunt – vancomycin + cefta
 OM, mastoiditis or sinusitis – vanco + 3rd gen ceph + metro
 duration of therapy – 4-6 wks

 Indications of surgery:
1. Abscess larger than 2.5 cm in dm
2. Gas is present in the abscess
2. Multiloculated lesion
3. Lesion is located in the post fossa
4. Fungus is identified

PSEUDOTUMOR CEREBRI
 clinical syndrome that mimics brain tumors & is characterized by increased ICP w/ normal CSF cell count & CHON
content & normal ventricular size, anatomy & position

 Etiology :
 alterations in CSF absorption & prodn, cerebral edema, abnormality in vasomotor control & CBF & venous
obstruction
 metabolic disorders : galactosemia, hypoparathyroidism, prolonged corticosteroid tx or too rapid
withdrawal, vitamin A def, obesity, menarche, oral contraceptives & pregnancy, hypervitaminosis A,
Addison disease
 infectious causes : roseola infantum, chronic OM, mastoiditis, GBS
 drugs : nalidixic acid, tetracycline, nitrofurantoin, isotretinoin, doxycycline, minocycline
 hema dso : polycythemia, hemolytic & Fe def anemia, Wiskott-Aldrich syndrome
 other causes: head injury, obstruction of SVC

 Clinical manifestations:
 headache (most frequent sx)
 diplopia 2 paralysis of CN 6
 most px are alert
 infants – bulging fontanel, (+) macewen sign
 papilledema w/ enlarged blind spot – most consistent sign in a child beyond infancy

 Treatment :
 discovery & tx of underlying cause
 mainly a self-limited condition
 initial LP that follows CT/MRI is dx & therapeutic
 Acetazolamide 10-30 mg/kg/d & corticosteroids have been effective for some px

SPINAL CORD DISORDERS

A. SPINAL CORD TUMORS

 account for approx 20% of neuroaxial tumors
 Intramedullary tumors – arise w/in substance of SC, grow slowly by infiltration, usually in cervical region

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  Most common intramedullary tumor : low grade astrocytoma

 Extramedullary intradural tumors

 benign & arise fr neural crest tissue
 examples : neurofibroma, ganglioneuroma, meningioma

 Extramedullary extradural tumors

 metastatic lesion
 examples : neuroblastoma, sarcoma & lymphoma

 Clinical manifestations :
 gait disturbances, scoliosis, back pain
 extramedullary tumors – back pain is often presenting symptom, child has difficulty sleeping bec of pain &
maintains a tripod posture while attempting to assume a supine position
 extramedullary extradural tumors – flaccid paraplegia, urinary retention, patulous anus

BROWN-SEQUARD SYNDROME
 ipsilat weakness, spasticity & ataxia w/ contralateral loss of pain & temp sensation
 seen in some extramed tumors
 Diagnosis :
 MRI –most impt dx test
 Xrays
 Treatment :
 surgery
 radiation therapy

B. SC TRAUMA
 result from indirect trauma caused by hyperflexion, hyperextension or vertical compression accidents
 causes: traumatic breech deliveries, physical abuse (shaken baby syndrome), automobile & diving accidents, falls
& congenital defects ( DS )
 severe cord injury presents w/ spinal shock: flaccidity, areflexia & loss of sensation ; persists for 4 wks
 fracture dislocations at the C5-C6 level are the most common acute cause of SCI ; characterized by flaccid
quadriparesis, loss of sphincteric function & sensory level corresponding to the upper sternum
 fractures in T12-L1 region – produce “conus medullaris syndrome” w/c includes loss of urinary & rectal sphincter
control, flaccid weakness & sensory disturbance of legs
 “central cord lesion” may result from contusion & hemorrhage, involves UE to a greater degree than legs
 Treatment:
 stabilization & immobilization of spine at the accident using cervical collar or sandbags
 high dose methylprednisolone IV should be started stat even before transport. If steroids are started w/in 3-
8 hr of injury, should be continued for 48 hr

C. TETHERED CORD
 results when a thickened ropelike filum terminale persists & anchors the conus at or below L2 level
 infants may have asymmetric growth in a foot or leg assoc w/ talipes cavus deformities & muscle wasting

D. SYRINGOMYELIA :
 a cystic cavity w/in the SC that may communicate w/ CSF pathways or remain localized & noncommunicating
 a rapidly progressive scoliosis may be the initial manifestation
 MRI study of choice
 surgical mgt

E. TRANSVERSE MYELITIS
 charac by abrupt onset of progressive weakness & sensory dist in LE
 (+) hx of preceding viral infx w/ fever & malaise
 caused by EBV, herpes, influenza, rubella, mumps, varicella
 Clinical manifestations:
 low back pain, abd pain, paresthesias of legs (early stages)
 pain, temp, & light touch sensation are affected but joint position & vibration sense are preserved
 (+) sphincter dist
 neuro deficit evolves for 2-3 days & plateaus, w/ flaccidity changing to spasticity
 spontaneous recovery occurs over a pd of wks or mos & is complete in approx 60% of cases
 residual deficits include bowel & bladder dysfx & weakness in LE

Ma. Enrica M.Pattaguan MD 2-A Page 15