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UMNL
DAYNA RYAN, PT, DPT
WINTER 2012
GENERAL TERMINOLOGY
PNS: motor units and associated sensory connections
Includes cranial nerves
CNS: brain and spinal cord
Sign = an objective finding revealed upon physical
examination
Symptom = (subjective) functional components of a disease
perceived by a patient and expressed in a patients history
Bells Palsy
Guillain-Barre
Charcot-Marie-Tooth Disease
Polio and post-polio syndrome
Diabetic polyneuropathy
Alcoholic polyneuropathy
Hansens Disease (leprosy)
MUSCLE HYPERACTIVITY
Fasciculations = rapid, fine, painless contraction of groups
of muscle fibers
Visible but not strong enough to move limbs
Commonly seen in anterior horn cell disorders=motor cells
(e.g. ALS)
Cramps = painful muscle spasms
When violent, referred to as convulsions
Clonic: alternating contraction and relaxation
Tonic: sustained contraction=charley horses
Also associated with anterior horn cell disorders
TETANY
= involuntary muscle twitching, spams, or cramps occurring
as a result of a hyperexcitable peripheral nerve
**can also occur in CNS disorders
Fibrillation: small, asynchronous contractions occurring
in a single skeletal muscle fiber
Too small to see (need an EMG to detect)
Present following denervation and with some myopathies
Myotonia: delayed capacity for relaxation in skeletal
muscle (tone disorder)
Contracture: tightness or restricted ROM across a joint
(common for Parkinson's, due to basal ganglia)
LMNL CHARACTERISTICS
Muscle Fatigability (myasthenia)
Decreased muscle endurance because cannot recruit
because of lack of innervation
Hypotonia: decreased muscle tone
Increased risk of subluxation (shoulders, scoliosis)
Abnormal Sensation
Often an early presenting sign of PNS dysfunction
Paresthesias = tingling or pins and needles sensations
Decreased or absent DTRs
Due to disrupted reflex arc (motor or sensory path)
Myopathies exhibit this because muscle cannot respond to
the nerve impulse
SENSORY ABNORMALITIES
THAT ACCOMPANY LMNLS
Anesthesia: Loss of feeling or sensation,
especially the loss of pain.
Hypesthesia: Decreased sensitivity to
sensory stimulation; also called hypoesthesia.
Paresthesia: Abnormal sensation such as
burning, pricking, tickling, or tingling,
especially occurring spontaneously.
Hyperesthesia: Exaggerated unpleasant
sensitivity to touch or other non-noxious
sensory stimuli.
SENSORY ABNORMALITIES
THAT ACCOMPANY LMNLS
Hypalgia: Diminished sensitivity to painful
stimuli:; also called hypoalgia.
Hyperalgia: Excessive sensitivity to painful
stimuli.
Causalgia: sensation of persistent, severe
burning of the skin, often accompanied by
hypersensitivity to touch and temperature
and trophic skin changes
Fredericks &
Saladin book
Pathophysiol
ogy of the
Motor
Systems
BRAIN STEM
Lesion
Upper midbrain
Posteriolateral medulla or
lower pons
Spinalthalamic,
Contralateral face affected
from second order neurons
from CNV
Contralateral in body.
Proprioception is ipsilateral
in face (only the CNVprop
fibers have not crossed at
this level).
BRAINSTEM LESIONS
BRAINSTEM LESIONS
BELLS PALSY
Innervation to
upper face is
bilateral
Innervation to
lower face is
unilateral (from
opposite
hemisphere)
a is Bells Palsy
b is stroke
Person in a and b
requested to
smile and close
eyes
BELLS PALSY:
ETIOLOGY
Unknown in most cases
May be secondary to viral infection causing
swelling in auditory canal or exposure to cold
temperatures
In a small number of cases, secondary to
acoustic neuroma impinging on nerve
Sensory:
Decreased taste on
ipsilateral tongue
ANS:
Decreased tearing (dry
eye)
Characteristic Smile
BELLS PALSY:
INCIDENCE AND ONSET
Incidence is 20/100,000 in US each year (affects
20,000-100,000 people in US / year)
Typical onset is overnight
Onset more common between 20-40 y.o.
(increased incidence with increased age)
Increased risk in diabetics and pregnant women
and people with MS
BELLS PALSY:
MEDICAL TREATMENT
High-dose corticosteroids for 5 days followed by a
tapered dose for another 5 days
Antiviral medications, e.g. acyclovir
Because of positive association between HSV and
Bells Palsy
Improves outcomes when paired with corticosteroids
DIABETIC
POLYNEUROPATHY
Affects PNS axons primarily (some demyelination)
Etiology: disrupted microcirculation
Onset:
After long duration diabetes
In diabetics who have had diabetes for 25+ years, 50% have
this condition
DIABETIC
POLYNEUROPATHY
Characteristics
Large nerve fiber involvement (most common)
painless paresthesias in feet and lower legs
decreased vibration and proprioception sense
decreased DTRs
DIABETIC
POLYNEUROPATHY
Screening/Examination Tests
NCVs
Monofilament screening with 5.07 / 10 gm. filament
Vibration
Complications
Diabetic ulcers (reducing WB helps)
50% of non-traumatic amputation in US are in diabetic patients
ALCOHOLIC
POLYNEUROPATHY
Affects PNS axon and myelin
Characteristics
Distal muscle weakness and atrophy
Sensory involvement greater in LEs than in UEs
Pain and paresthesias in distal legs and soles of
feet (this does not typically happen in the diabetic
polyneuropathy)
Aching in calf muscles
Decreased ankle reflexes and poor sensation in
feet
ALCOHOLIC
POLYNEUROPATHY
Cause
Insidious onset and slow progression
Exacerbations may occur
Sensory signs and symptoms before motor
Mild case = mild aching in calf and feet
Severe case = severe motor and sensory signs
Prognosis
with abstinence and improved nutrition, a slow,
incomplete recovery may occur
considerable discomfort may remain
STOCKING-GLOVE PATTERN OF
SENSORY IMPAIRMENT
CHARCOT-MARIE-TOOTH (CMT)
DISEASE
Also known as:
Hereditary Motor and Sensory Neuropathy Type I (HMSN)
OR
Peroneal Muscular Atrophy
Lesion site = PNS axon and PNS demyelination, anterior
horn cell and dorsal root ganglion
Onset
Hereditary
Late childhood or adolescence for CMT1
Adulthood for CMT2 (less severe with minimal sensory
loss)
Slowly progressive
CHARCOT-MARIE-TOOTH (CMT)
DISEASE
Characteristics
Pes cavus foot deformity (high arch) and hammer
toes
Symmetric weakness and atrophy in intrinsic foot,
peroneal, and anterior tibialis muscles
Weakness and atrophy rarely go above knee or
elbow
Paresthesias are common
Lead to foot drop (see steppage gait pattern)
Normal lifespan most remain ambulatory throughout
their lives
CHARCOT-MARIE-TOOTH
DISEASE
Foot deformity: high arch pes cavus foot deformity with
hammer toes
TABES DORSALIS
Lesion site: dorsal roots & posterior columns of lumbosacral
and lower thoracic S.C.
Etiology: From syphilis
Diagnosis based on:
Neuroimaging is normal
Abnormal tibial sensory NCVs
CSF has elevated protein level
Significant sensory loss!
TABES DORSALIS
Characteristics
Unilateral or bilateral presentation
Diminished proprioception and vibration in legs
Sensory ataxia of gait
Areflexia
Touch, pain, and temperature sensation are intact
Aching / paresthesias (radicular distribution)
May also have:
Autonomic disturbances (bladder atony(incontinence),
papillary abnormalities, impotence)
Later onset of distal muscle weakness and atrophy (if
anterior horn cells become involved)
HANSENS DISEASE
(LEPROSY)
Lesion site: Peripheral
nerves
Etiology: Mycobacterium
leprae infection
Two forms:
Tuberculoid
Primarily superficial nn
Leprous
Weakness - symmetric
Sensory loss
Skin lesions
GUILLAIN-BARRE
SYNDROME (GBS)
Alternate Name: Acute Inflammatory
Demyelinating Polyradiculoneuropathy = AIDP
Many variants of the syndrome exist
Lesion site
PNS myelin (secondary axonal degeneration in
many patients)
Etiology
autoimmune disorder with unknown trigger (may be
secondary to an upper respiratory or GI infection)
GUILLAIN-BARRE SYNDROME
(GBS)
Primary Characteristics
Weakness
typically symmetric, beginning in distal LE muscles and
ascending through body rapidly including facial muscles
facial and palatal weakness is common ( in about 50% of
cases)
GUILLAIN-BARRE SYNDROME
(GBS)
Early symptoms
difficulty with walking
paresthesia in toes (commonly 1st symptom)
muscle tenderness (tender to touch)
About 50% have respiratory involvement
up to 30% of GBS patients require mechanical ventilation
during the acute phase
Dysarthria=slurring of words, dysphagia=difficulty
swallowing, and diplopia=double vision develop in severe
cases
GUILLAIN-BARRE SYNDROME
(GBS)
Can occur at any age, but mostly 5th-8th decade
Disease course:
Maximal onset in less than 4 weeks, many in a few days
Static phase (plateau of 2-4 weeks)
Recovery takes months to years
Recurs in 10% of cases
Static Phase
Recovery
Onset
Weeks
GUILLAIN-BARRE SYNDROME
(GBS)
5% mortality rate
At 6 months, 85% are ambulatory
At 1 year, 20% remain significantly handicapped by
weakness
At 2 years, 8% have not achieved full recovery
Poorer prognosis with:
Etiology
Polio virus initially.
Existing motor neurons reduce
the number of collateral sprouts
resulting in more muscle cell
death.
As motor neurons die with age,
there is no redundancy in the
system for other motor units to
take over, so motor function is
decreased.
NOT due to reactivation of the
polio virus.
Motor
Paresis or flaccid
paralysis
Decreased or absent
DTRs=lmn
Decreased endurance for
physical activity
Exercise recommendations:
Never exercise to point of
fatigue (use general body
conditioning and low resistance
exercises)
Monitor vitals before and after
exercise (remember that
respiratory muscles are also
affected)
Caution patient to stop exercise
if pain persists or weakness
increases
Post-polio S & S
Decreased
strength in
previously affected
muscles
New muscle
weakness
Increased muscle
atrophy
Myalgia
Joint pain
AMYOTROPHIC LATERAL
SCLEROSIS (ALS)
Also called Lou Gehrigs
Disease
Lesion site: anterior horn
cells, lateral
corticospinal tract, motor
nuclei of brainstem, and
motor area of frontal lobe
(pre-central cortex)
Etiology unknown except
in a few inherited cases
AMYOTROPHIC LATERAL
SCLEROSIS (ALS)
LMN signs
Progressive muscle wasting
Weakness (asymmetric weakness is often presenting
sign)
Fasciculations (especially evident in tongue)
cramps
UMN signs
Spasticity
Hyperreflexia
Positive Babinski
AMYOTROPHIC LATERAL
SCLEROSIS (ALS)
Most common to see:
AMYOTROPHIC LATERAL
SCLEROSIS (ALS)
Prognosis
Death in 2-5 years commonly from respiratory
compromise (mean death is 3-4 years after onset if
patient does not get ventilatory support)
20% survive more than 5 years
those who have ALS before age 50 generally live
longer
AMYOTROPHIC LATERAL
SCLEROSIS (ALS)
Functions typically preserved throughout disease:
Intellect is not affected
Eye movements
Bowel and bladder control