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203 Cards in this Set
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What does lack of nystagmus in setting of vertigo indicate?
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Lesion is central. Peripheral lesions always are associated with nystagmus
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Which structures when damaged may produce cerebellar ataxia?
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cerebellar peduncles
red nucleus pons spinal cord cerebellum Unilateral frontal disease occasionally mimics disorder of conralateral cerebellar hemisphere |
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What is asynergia and ewhen does it occur?
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complex movements become decomposed into succession of indivudual movements rather than single smooth motor act
cerebellar ataxia |
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What are the midline structures of the cerebellum?
What is their function? |
Vermis, flocculonodular lobe, fastigial nuclei
control of axial functions - eye movements, head and trunk posture, stance, gait nystagmus, oscillation of head and trunk, instability of stance, gait ataxia. |
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Syndrome resulting from cerebellar hemispheric lesion?
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ipsilateral hemiataxia, hypotonia of limbs, nystagmus and transient ipsilateral gaze paresis
Paramedian lesions in left cerebellar hemisphere cauess cerebellar dysarthria |
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Tracts through the superior cerebellar peduncle?
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Afferent
Ventral spinocerebellar Efferent Cerebellothalamic Cerebellorubral Cerebelloreticular |
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Tracts through middle cerebellar peduncle?
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Afferent
Corticopontocerebellar |
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Tracts through inferior cerebellar pednuncle?
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Afferent
Vestibulocerebellar Cuneocerebellar Nucleocerebellar REticulocerebellar Oolivocerebellar Arcuatocerebellar Dorsal spinocereellar Efferent Cerebellovestibular |
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Differential for orthostatic hypotension in setting of sensory ataxia?
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Tabes dorsalis
polyneuropathies spinocerebellar degeneration |
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Gait in cerebellar ataxia?
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wide based, staggering, oscillation of trunk or head
deviation towards side of lesion if unilateral hemispheric lesion with eyes closed |
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Gait in sensory ataxia?
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wide based, tandem poor
lifting feet high off the ground and slapping them down heavily - steppage gait |
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Nystagmus in peripheral vestibular disorder?
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jerk nystagmus maximal on gaze away from involved side
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Direction of nystamus in cold calorics?
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fast phase away from irrigated ear, slow phase towards ear.
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What procedure should not be done in suspected cerebellar hemorrhage?
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lumbar puncture
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What serological abnormalities accompany ataxia telangiectasia?
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elevated AFP
immunoglobulin deficiency |
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In what cases and for what purposes is audiometry useful?
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vestibular disorders associated with auditory impairement
can seperate conductive, labyrinthine, acoustic nerve, and brainstem disease |
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What is the most common cause of peripheral vertigo?
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BPPV - 30%
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In which position or BPPV attacks the worst?
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lateral decubitus with affected ear down.
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Typical course of BPPV?
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episodic vertigo x weeks with spontaneous resolution
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Describe repositioning maneuver for BPPV
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head is turned 45 degrees in direction of affected ear while sitting
Pt then lays supine with head hanging down over end of examining table Head is turned 90 degrees in opposite direction Pt then rolls to lateral decubitus with affected ear up pt turns to prone position nad sits up |
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When does meniere's disease have its onset?
Gender distribution |
20-50 years of age
M>F |
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Treatment of Meniere's?
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diuretics like HCTZ, triamterene
Antihistamines, anticholinergics, benzodiazepines, sympathomimetics endolymphatic shunting, labyrinthectomy, vestibular nerve section in refractory cases |
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What are the drugs used in the treatment of vertigo?
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Antihistamines
Meclizine, Promethazine, Diphenhydramine Anticholinergics Scopolamine Benzodiazepines Diazepam Sympathomimetics Amphetamine, Ephedrine |
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What is acute peripheral vestibulopathy?
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Spontaneous attack of vertigo of inapparent cause that resolves spontaneously and is not accompanied by hearing loss or evidence of CNS dysfunction
Includes acute labrythitis, vestibular neuronitis |
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How long do attacks of acute peripheral vestibulopathy last?
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up to 2 weeks, symptoms may be recurrent, some degree of dysfunction may be permanent
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Presentation of attack of acute peripheral vestibulopathy?
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Patient ill appearing, lays with affected ear upward, reluctance to move head.
Nystagmus with fast phase away from affected ear |
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Treatment of acute peripheral vestibulopathy?
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Treatment with 10-14 day course of prednisone 20 mg and/or general vertigo treatments
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Presentation of otosclerosis?
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conductive hearing loss, sensorineural hearing loss and vertigo are common, tinnitus is infrequent
Auditory symptoms begin vbefore 30 years Recurrent episodic vertigo with or without positional vertigo and sense of positional imbalance |
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Features favoring diagnosis of otosclerosis over meniere's disease?
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positive family history
tendency towards onset at earlier age presence of conductive hearing loss bilateral symmetric auditory impairment |
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Treatment of otosclerosis?
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sodium fluoride, calcium gluconate, vitamin D
If refractory, surgical stapedectomy |
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What are common tumors at cerebellopontine angle?
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acoustic neuroma, meningiomas, cholesteatomas (epidermoid cysts)
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Features of NF1?
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cafe-au-lait spots, cutaneous neurofibromas, axillary or inguinal freckles, optic gliomas, iris hamartomas, dysplastic bony lesions
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Features of NF2?
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bilateral acoustic neuromas, other tumors of central or peripheral systems including neurofibromas, meningiomas, gliomas, schwannomas
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Which nerves are typically affected by tumors of the cerebropontine angle?
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VII, VIII, V
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In a patient with cerebellopontine angle tumors what features suggest meningioma? Cholesteatoma?
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Meningioma - isolated acoustic nerve disease
Cholesteatoma - conductive hearing loss, early facial weakness, or facial twitchign with normal CSF protein |
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Clinical features of alcoholic vestibulopathy?
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Worst as the person becomes intoxicated and then again with recovery due to differing concentrations in the cupula and endolymph.
Syndrome starts ~2 hrs after ingesion lasts up to 12 hours and consists of twyo symptomatic phases seperated by 1-2 hour asymptomatic interval vertigo and nystagmus in lateral recumbent position, accentuated with eyes closed Spontaneous nystagmus, dysarthria, gait ataxia from cerebellar dysfunction |
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How do aminoglycosides cause hearing loss?
Which aminoglycosides are more often implicated |
destruction of hair cells
streptomycin, gentamicin, tobramycin cause vestibular toxicity amikacin, kanamycin, tobramycin most likley to cause hearing loss |
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What vascular territories when affected by ischemia cause vertigo?
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Intrenal auditory artery - supplies acoustic nerve, originates from basilar or atnerior inferior cerebellar artery
Lateral medullary infarction - proximal vertebral artery occlusion most often Superior, anterior inferior, posterior inferior cerebellar arteries paramedial midbrain infarction - paramedian penetrating branches of basilar artery |
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Describe the syndrome caused by internal auditory artery occlusion
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nystagmus and vertigo with fast phase directed away from involved side - unilateral hearing loss, sensorineural
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Describe the syndrome caused by lateral medullary infarction
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vertigo, nausea, vomiting, dysphagia, hoarseness, nystagmus,
Ipsilateral - Horner syndrome, limb ataxia, impairment of all sensory modalities over face, loss of light touch and position sense in limbs Contralateral - impairment of pinprick and temperature sense in limbs Vertigo is from vestibular nuclei involvment, hemiataxia from inferior cerebellar peduncle involvement |
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Describe Benedikt syndrome
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ipsilateral medial rectus palsy, fixed dialted pupil
contralateral limb ataxia affecting only the arm typically cerebellar signs from involvement of red nucleus which receive crossed projection from superior cerebellar peduncle |
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What neurologic deficit is associated with legionella?
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cerebellar syndrome without clinical evidence of meningitis
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What part of the cerebellum typically degenerates in alcoholic cerebellar degeneration?
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superior vermis is affected preferentially
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Time course for alcoholic cerebellar degeneration?
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Progression over weeks to months most common compared to years
Sometimes abrupt onset ataxia |
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Clinical picture in alcoholic cerebellar degeneration?
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Gait ataxia is a universal feature
distal sensory deficits in feet and absent ankle reflexes from polyneuropathy signs of malnutrition - loss of subcutaneous tissue, generalized muscle atrophy, glossitis |
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What areas of cerebellum are most affected in phenytoin induced cerebellar degeneration?
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inferior and posterior vermis is affected most severely, superior vermis is relatively spared.
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Clinical syndrome produced by phenytoin induced cerebellar degeneration?
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nystagmus, dysarthria, ataxia affecting limbs, trunk, and gait
Polyneuropathy sometimes Symptoms typically irreversible |
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Cerebellar degeneration is associated most frequently with which cancers?
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ovarian cancer, lung cancer (small cell especially), Hodgkin disease, breast cancer
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Clinical syndrome produced by paraneoplastic cerebellar degeneration?
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gait and limb ataxia most prominent, dysarthria in most cases, involvment is often assymetric
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For each cancer name paraneoplastic antibodies most closely associated:
Breast Lung Ovarian |
Breast - anti-Yo, Anti-Ri
Lung - Anti-Hu Ovarian - Anti Yo |
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What clinical features can be used to seperate paraneoplastic cerebellar degeneration from that caused by hypothyroidism or alcoholism?
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dysarthria and dysphagia. Also ataxia of the arms suggests alcohol is unlikely cause
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What are the more common genetic syndromes associated with cerebellar atrophy?
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Spinocerebellar ataxia - trinucleotide repeat disease (also 5 nucleotide sequence repeat with anticipation)
Dentatorubral pallidollysian atrophy - also AD cerebellar ataxia. Polyglutamine expansion. Friedreich ataxia - trinucleotide repeat on chromosome 9 Ataxia-Telangiectasia - AR with onset in infancy from ATM gene mutation - defect in DNA repair Wilson disease - autosomal recessive from ATP7B gene CJD |
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Which areas degenerate in Friedrich's ataxia?
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spinocerebellar tracts, posterior columns, dorsal roots, depletion of neurons in Clarke column (dorsal spinocerebellar tracts), Large myelinated axons of peripheral nerves and cell bodies of primary sensory neuronsi n dorsal root ganglia are also involved.
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Clinical findings in Friedrich's ataxia?
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Always occurs after 4 years of age and before end of puberty
Progressive gait ataxia, then ataxia of all limbs within 2 years. Knee and ankle tendon reflexes are lost, cerebellar dysarthria appears. Reflexes in arms and sometimes in knees are preserved. Joint position, vibration sense are imparied in legs adding senory component to gait ataxia extensor plantar responses, pes cavus Cardiomyopathy, scoliosis leading to restrictive lung disease, ocular atrophy, nystagmus, paresthesias, tremor, hearing loss, vertigo, spasticity, leg pains, diabetes mellitus |
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Course of Friedrich's ataxia?
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inability to walk unaided in 5 years after symptom onset, bedridden state in 10-20 years
average duration of sympomatic illness is 25 years with death at 35 typically |
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Clinical presentation of ataxia telangiectasia?
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progressive pancerebellar degneration - nystagmus, dysarthria, gait, lumb, truncal ataxia begining in infancy.
Choreoathetosis, loss of vibration and position sense in legs, areflexia, disorders of voluntary eye movement are almost always universal findings. Mental deficiency in second decade. Oculocutaneous telangiectasia in teen years. Bulbar conjunctivae affected first then sun-exposed areas of skin. vascular lesions don't bleed, spread to CNS. Immunologic impairment - Decreased IgA, IgE, recurrent sinopulmonary infections progeric changes in skin and hair, hypogonadism, insulin resistance |
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Lab findings in ataxia telangiectasia?
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decreased IgE, IgA, increased alphafetoprotein, CEA
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What features seperate ataxia telangiectasia from Friedrich's ataxia?
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Ataxia-telangiectasia have onset before 4 years, choreoathetosis associated, absence of skeletal abnormalities such as kyphoscoliosis
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Differential diagnosis for light near dissociation?
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neurosyphillis, DM, optic nerve disorders, tumors compressing midbrain tectum, lesions in region of Edinger Westphal nucleus (MS)
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What findings do Argyll robertson pupils have in addition to light near dissociation?
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irregular in shape, unequal in size
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What is the eponym for tonic pupil?
What condition is it most commonly associated with and what are other signs? |
Adie pupil
Holes Adie syndrome - depressed deep tendon reflexes in legs, segmental anhidrosis, orthostatic hypotension, cardiovascular autonomic instability. |
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What causes pupillar abnormality in Holmes Adie syndrome?
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degeneration of ciliary ganglion with aberrant reinnervation of pupilloconstrictor muscles
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Path of the oculosympathetic fibers?
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Hypothalamus projecting through interomediolateral column of spinal cord at T1,
preganglionic sympathetic neuron from spinal cord to superior cervical ganglion postganglionic sympathetic neuron from superior cervical ganglion in neck along carotid and entering orbit with V1 |
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What is a sign of Horner's syndrome being present since infancy? What is the formal name for this sign?
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Blue ipsilateral iris (heterochromia iridis)
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What pathways are tested in the optokinetic response?
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slow pursuit tests ipsilateral parietoociipital pathways, rapid moevement tests pathways originating in contralateral frontal lobe
Test is useful in documenting visual perception in newborns or in psychogenic blindness |
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Finding on optokinetic response testing in unilateral parietal lobe lesion?
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impairment when target is moved towards the side of the lesion
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Differential for unilateral ptosis?
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Paralysis of levator palpebrae, lesions of CN 3, Horner's syndrome
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How do you differentiate horner's syndrome ptosis from CN3 ptosis?
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horner's syndrome ptosis can be overcome by effortful eye opening
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Differential for bilateral ptosis?
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Oculomotor nerve nucleus
Disorder of neuromuscular junction Disorder of muscle - myotonic, ocular, oculopharyngeal dystrophy |
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Causes of lid retraction?
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Parinaud's syndrome, Graves
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Causes of exopthalmos?
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hyperthyroidis, orbital tumor or pseudotumor, carotid artery cavernous sinus fisutula
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How do people in coma with intact brainstem respond to cold calorics unilaterally? bilaterally?
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Unilateral - deviation towards cold
Bilateral - downward deviation |
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What does nonresponse to calorics typically indicate?
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peripheral vestibular disease, structural lesion in posterior fossa, intoxication with sedative drugs
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Testing for trochlear nerve lesion?
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elevation greatest when head is tilted toward involved side, abolished by tilt in opposite direction
Diplopia most pronounced when looking down with eye affected adducted |
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How can you seperate gaze palsies on physical exam from other deficiencies in ocular movement?
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calorics
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What causes one and half syndrome?
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lesion involving medial longitudinal fasiculus and ipsilateral PPRF
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What are the different types of nystagmus?
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Pendullar - onset in infancy - qual in volocity in both directions
Jerk - slow phase of movement followed by a fast phase in opposite direction. Direction of the fast phase specifies direction of nystagmus |
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What is the most common cause of multidirectional gaze evoked nystagmus?
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anticonvulsant or sedative drugs
cerebellar or central vestibular dysfunction can cause as well |
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What typically causes nystagmus evoked by gaze?
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early or mild residual ocular palsy
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How does vestibular nystagmus present?
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Nystagmus increasing with gaze toward the fast phase.
Peripheral - increases with gaze toward the fast phase Central - bidirectional and purely horizonatal, vertical, or rotatory and with mild vertigo |
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What are causes of papilledema outside intracranial hypertension?
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congenital cyanotic heart disease, increased CSF protein content (spinal cord tumor and idiopathic inflammatory polyneuropathy)
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Midbrain lesions cause what sorts of vision abnormalities?
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upgaze paralysis, some or all of Parinaud syndrome, perserved reflex of vertical eye movements with doll's head maneuver or Bell phenomenon (elevation of eye with eyelid closure), nystagmus (esp on downward gaze and associated with retraction of eyes), paralysis of accommodation, midposition pupils, light-near dissociation
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How does Benedikt syndrome present?
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oculomotor palsy and contralateral ataxia
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How does Weber's syndrome present?
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oculomotor palsy and contralateral hemiplegia
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What does abducens palsy in absence of increased intracranial pressure responding to prednisone therapy indicate?
What should be done in case pred does not work? |
superior orbital fissure syndrome or tolosa hunt syndrome
Do CT for cavernous sinus involvement |
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What are the progressive external ophthalmoplegias?
What causes these syndromes? |
syndromes characterized by slowly progressive symmetric impairment of ocular movement that cannot be overcome by caloric stimulation
Preserved pupillary function, no pain, ptosis often prominent ocular or oculopharygneal muscular dystrophy |
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What does progressive external opthalmoplegia associated with myotnic contraction on percussion of muscle groups indicate?
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Myotonic dystrophy
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What is typicallly cause of Kearns Sayre Daroff syndrome?
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deletion of muscle mitochondial DNA
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Features of Kearns Sayre Daroff syndrome?
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progressive external ophthalmoplegia with pigmentary degeneration of retina, cardiac conduction defects, cerebellar ataxia, elevated CSF protein
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Diagnosis of Kearns Sayre Daroff syndrome?
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muscle biopsy with ragged red fibers
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What are causes of hypotonia?
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disruption of afferent limb of reflex arc
particular extrapyramidal disorders like Huntingtons acute stage of pyramidal lesions primary muscle disorders |
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What causes lead pipe rigidity?
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basal ganglia lesion
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What causes paratonia?
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diffuse cerebral disease, frontal lobe lesion
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What are tests of rapid alternating movement?
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tapping repetitively with on hand on the back of the other
tapping alternatveily with palm and back of one hand on the back of hte other hand or knee screwing an imaginary light bulb into the ceiling with each arm in turn rubbing fingers of one hand in circular polishing movement on the back of hte other hand tapping on ball of the thumb with the tip of the index finger tapping the floor as rapidly as possibly with sole while keeping heel in place |
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How are tests of rapid alternating movements affected by pyramidal lesions?
cerebellar lesions? |
pyramidal - fine voluntary movements are performed slowly
cerebellar - rate, rhythm, amplitude is irregular |
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What nerve roots subserve cremesteric reflexes?
In what conditions in which this reflex arc is not lost is the cremesteric reflex lost? |
L1, L2
contralateral upper motor neuronal losses |
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In whom does apraxic gait tend to occur?
Describe gait? |
Patients with frontal lobe dysfunction (typically bilateral)
No weakness or incoordination, patient is unable to stand unsupported or to walk properly with feet appearing glued to ground. When patient can manage to walk gait is short stepped, with marked hesitation, legs moved in direction inappropriate to center of gravity. |
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What is asynergia or dyssynergia?
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breakdown of complex actions into individual movements composing them
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What are pharmacologic options for treatment of spasm?
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diazepam, baclofen, dantrolene, dizanidine
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Progression of disease from total spinal cord resection?
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Initially flaccid paralysis with loss of tendon and otehr reflexes with senory loss and urinary and fecal retention
Over weeks reflexes returns with spastic paraplegia - feces and urine are expelled at intervals. |
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What medical therapy should be used acutely in setting of spinal cord injury?
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methylprednisolone infusion - improves motor and sensory function at 6 months
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In what setting do MS exacerbations tend to occur?
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infection
3 months after childbirth rise in body temperature |
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Side effects of glatiramer acetate?
brand name? |
erythema at sites of injection
transient episodes of flushing ,dyspnea, chest tightness, palpitations, anxiety Copaxone |
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What can be used to treat fatigue in MS?
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Amantadine, SSRI
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Which viral infections are most likely to precipitate ADEM?
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measles, chickenpox
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Where does vacuolar myelopathy most often found?
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white matter of spinal cord most prominantly in lateral, posterior columns
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What are common causes of myelopathy in AIDs?
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Vacuolar myelopathy
Lymphoma Cryptococcal infection Herpesviruses |
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What typically accompanies vacuolar myelopathy?
What are symptoms of vacuolar myelopathy? Exam findings? |
AIDS dementia complex
leg weakness, ataxia, incontinence, erectile dysfunction, paresethesias, paraparesis, lower extremity monoparesis, quadriparesis, spasticity, increased or decreased tendon reflexes |
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What spinal cord manifestation is caused by HTLV-1?
Where does this disease typically occur? |
tropical spastic paraparesis
Caribbean, off Pacific coast of columbia, in Seychelles |
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Clinical features of tropical spastic paraparesis?
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spastic paraparesis, impaired vibration and joint position sense, bowel and bladder dysfunction
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What viral syndromes may produce myelopathies?
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HIV
CMV HSV 1,2 Varicella HTLV-1 |
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In what clinical setting does chronic adhesive arachnoiditis occur?
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follows SAH, meningitis, intrathecal administration of penicillin, radiologic contrast material, spinal anesthetics of certain sorts, trauma, surgery
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How does chronic adhesive arachnoiditis maniest?
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Constant radicular pain,
sometimes lower motor neuron weakness from involvement of anterior nerve roots spastic ataxic paraparesis with sphincter involvement |
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Which are more common and by how much: intramedullary spinal cord tumors or extramedullary?
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Extramedullary are 90%, Intramedullary 10%
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What is the most common intramedullary spinal cord tumor?
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Ependymomas
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What are the most common primary extramedullary spinal cord tumors?
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Neurofibromas, meningiomas
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What types of cancers most commonly produce extramedullary spinal cord compression?
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bronchus, breast, prostate, lymphomatous, leukemic deposits, myeloma
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What is the Queckenstedt test and what was it used for?
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Patient lays in lateral decubitus position and has lumbar puncture with pressure measurement
With needle in examiner presses on both jugular veins engorging CNS vasculature causing rise measured pressure if no spinal block |
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Eponym for SMA I?
Inheritance? Clincial features? Clinical course? |
Werdnig Hoffmann
AR Floppy infant with difficulty sucking, swallowing, ventillation. Atrophy and fasciculation of tongue, muscle wasting in the limbs Rapidly progressive with death from respiratory complications by 3 years |
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Eponym for SMA II?
Inheritance? Clincial features? Clinical course? |
Chronic Werdnig Hoffmann Disease
AR begins in latter half of first year, wasting, weakness of extremiteis, bulbar weakness less common slow progression with kyphoscoloiosis and contractures. Many survive to adulthood |
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Eponym for SMA III?
Inheritance? Clincial features? Clinical course? |
Kugelberg Weldander Disease
AR, sometimes sporadic develops in childhood or early adolescence proximal limb muscle involvement with little involvment of bulbar musculature Progressive course with disability in early adult life. |
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What are the types of motor neuron disease presenting in adults and their features?
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Progresssive bulbar palsy - bulbar involvement predominates and is due to lesions affecting motor nuclei of cranial nerves in brainstem
Pseudobulbar palsy - bulbar involvement predominates and is due to upper motor neuron disease Progressive spinal muscular atrophy - lower motor neuron deficit in limbs from anterior horn cell degeneration Primary lateral sclerosis - purely upper motor neuron deficit is found in limbs Amyotrophic lateral sclerosis - mixed upper and lower motor neuron deficit in limbs, sometimes bulbar involvement of upper or lower motor neuron type |
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Presenting complaint in ALS by frequency?
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Weakness of upper extremity muscles (40%)
Weakness of lower extremity muscles (40%) Weakness of bulbar muscles (20%) |
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What muscles are typically spared in ALS?
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Extraocular muscles, sphincters
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Primary medication for ALS?
What does it do? |
Riluzole
Blocks glutaminergic transmission in CNS prolongs survival for 2-3 months |
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Prognosis for ALS?
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Most motor neuron disease is progressive and has fatal outcome in 3-5 years usually from pulmonary infections
Pts with bulbar involvement have worse prognosis than those with isolated limb abnormalities |
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What are noninfective disorders of anterior horn cells
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motor neuron disease of adults (ALS spectrum)
SMA I, II, III Kennedy syndrome hexosaminidase deficiency (Tay-Sachs) Monoclonal gammopathy sometimes produces pure motor syndromes Rare complication of lymphoma |
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How does anterior horn cell disease from lymphoma typically present?
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onset typically after diagnosis of lymphoma is established, weakness in legs, patchy in distribution, spares bulbar and respiratory muscles
Neurologic deficits progress over months are followed by spontaneous improvement and in some cases improvement |
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What is given for therapy for anterior horn cell disease secondary to monoclonal gammopathy?
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Plasmapheresis and immunosuppressive drug treatment with dex and cyclophosphamide
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What is Kennedy syndrome? Transmission, clinical features, prognosis?
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Bulbospinal neuronopathy
Sex linked recessive from trinucleotide repeat on androgen receptor More benign prognosis than other motor neuron diseases tremor resembling essential tremor, cramps, fasciculations, proximal weakness, twitching movements of chin that are precipitated by pursing of lips |
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What virus produces a syndrome similar to polio?
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coxsackie virus
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Which nerve roots are involved in Erb Duchenne Palsy?
In what context does this occur? |
C5, C6
birth trauma |
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In what context does Klumpke's paralysis occur?
Which nerve roots are involved? Clinical syndrome? |
C8, T1
fall arrested by grasping a fixed object with one hand, or traction on abducted arm paralysis and wasting of small muscles of hand and of long finger flexors and extensors Horner syndrome sometimes |
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How does neuralgic amyotrophy present?
What is another name for the syndrome? |
severe pain about shoulder followed within days by weakness, reflex changes, sensory disturbances in arm involving C5, C6 especially
Usually unilateral, sometimes bilateral Idiopathic brachial plexopathy |
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Presentation of cervical rib syndrome?
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C8, T1 involvement
compression by cervical rib or band arising from 7th cervical vertebra Weakness, wasting of intrinsic hand muscles - especially those in thenar eminence with accompanying pain and numbness in dermatomal distribution Sometimes compression of subclavian artery |
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What is the Adson test and what does it typically diagnose?
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decreased radial pulse on turning head.
Cervical rib syndrome |
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What are causes of brachial plexopathy?
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Neuralgic amyotrophy
Cervical rib syndrome Neoplastic infiltration Radiation therapy median sternotomy Trauma |
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What peripheral nerve lesion should be suspected if foot drop occurs during labor?
What causes it? Prognosis? |
Intrapartum maternal lumbosacral plexopathy
short women, relating to compression fo lumbosacral trunk by ftetal head at pelvic brim Recovery in 6 months |
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How do neurologic sequelae of diptheria infection typically manifest?
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palatal weakness 2-3 weeks after infection of throat
cutaneous diptheria may be followed by focal weakness of neighboring muscles after similar time period impaired pupillary responses to accommodation 4-5 weeks after infection generalized sensorimotor polyneuropathy after 1-3 months Weakness may be asymmetric and may be more marked proximally than distally sometimes respiratory paralysis recovery over 2-3 months but sometimes longer |
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How do neurologic sequelae of diptheria infection typically manifest?
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palatal weakness 2-3 weeks after infection of throat
cutaneous diptheria may be followed by focal weakness of neighboring muscles after similar time period impaired pupillary responses to accommodation 4-5 weeks after infection generalized sensorimotor polyneuropathy after 1-3 months Weakness may be asymmetric and may be more marked proximally than distally sometimes respiratory paralysis recovery over 2-3 months but sometimes longer |
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Treatment for diphtheria infection?
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early administration of equine diphtheria antitoxin without results of bacterial culture
2 week course of penicillin or erythromycin antibiotics do not alter incidence of serious complications |
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Treatment for diphtheria infection?
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early administration of equine diphtheria antitoxin without results of bacterial culture
2 week course of penicillin or erythromycin antibiotics do not alter incidence of serious complications |
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What is the toxin that is responsible for paralytic shellfish poisoning?
how does it present? Management? |
saxitoxin
sensory symptoms and rapidly ascending paralysis cathartic or enema to remove unabsorbed toxin |
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What is the toxin that is responsible for paralytic shellfish poisoning?
how does it present? Management? |
saxitoxin
sensory symptoms and rapidly ascending paralysis cathartic or enema to remove unabsorbed toxin |
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How does neuropathy secondary to porphyria typically manifest?
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Weakness due ot motor polyneuropathy causing symmeteric disturbance, sometimes more marked proximal than distal. May begin in upper limbs or trunk
Rapid progresssion with complete flaccid quadriparesis in days Sometimes sensory loss but less conspicuous and extensive |
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How does neuropathy secondary to porphyria typically manifest?
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Weakness due ot motor polyneuropathy causing symmeteric disturbance, sometimes more marked proximal than distal. May begin in upper limbs or trunk
Rapid progresssion with complete flaccid quadriparesis in days Sometimes sensory loss but less conspicuous and extensive |
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Diagnosis of porphyria?
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acute intermittent porphyria - increased levels of porphobilinogen and delta aminolevulinic acid in urine or deficiency of uroporphyrinogen I synthetase in red blood cells
hereditaory coproporphyria - increased coproporphyrinogen oxidase in lymphocytes |
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Diagnosis of porphyria?
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acute intermittent porphyria - increased levels of porphobilinogen and delta aminolevulinic acid in urine or deficiency of uroporphyrinogen I synthetase in red blood cells
hereditaory coproporphyria - increased coproporphyrinogen oxidase in lymphocytes |
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Treatment for porphyria?
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intravenous dextrose to suppress heme biosynthetic pathyway
propranolol to control tachycardia and hypertension Hematin by IV infusion once daily is effective in improving clinical state chlorpromazine or another phenothiazine may help abdominal and mental symptoms |
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How does arsenic or thallium toxicity present?
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Sensorimotor polyneuropathy with accompanying or preceding GI disturbance
Arsenic - skin rash with increased pigmentation, marked exfoliation, Mees lines in longstanding cases Thallium - scaly rash and hair loss Sensory symptoms are earliest mainfestation of polyneuropathy, followed by symmetric motor impairment more marked distally than proximal |
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Neurological symptoms related to organophosphate toxicity and their presentation?
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Delayed polyneuropathy - 1-3 weeks after acute exposure
Cramping muscle pain in the legs initially followed by distal numbness and paresthesias. Progressive leg weakness with depression of tendon reflexes. Similar deficits in upper limbs after several days. Sensory disturvances in some cases in legs first then arms - mild or inconspicuous Central deficits are permanent |
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Treatment of organophosphate toxicity?
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Bleach, soap to clean off skin
atropine 2-6 Q5 pralidoxime 1 g Q hour for up to 3 hours |
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Peripheral neuropathy in lead toxicity?
Therapy? |
Predominantly motor, more severe in arms than legs, typically affects radial nerves (mononeuropathy multiplex)
EDTA, pencillamine, dimercaperol |
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How does multifocal motor neuropathy typically present?
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Progressive asymmmetrical wasting and weakness, EMG/NCS evidence of multifocal motor demyelination with partial motor conduction block but normal sensory responses
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Lab test often positive in multifocal motor neuropathy?
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antiglycolipid antibodies in serum (anti-GM1 IgM)
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Clinical feaures which suggest multifocal motor neuropathy?
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motor deficit in distribution of two or more named nerves and related to conduction block outside common entrapment sites
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Treament of multifocal motor neuropathy?
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Cyclophosphamide once a month for 6 months (pred and plasmapheresis have been tried without success)
or IVIG over 3-5 days |
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What are populations in which bell's palsy occurs more commonly
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diabetics and pregnant women
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Course of treatment for Bell's palsy?
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corticosteroids (pred 60 for 3 days with tapering over a week
treat within 5 days of onset of palsy |
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What are disease associations for myasthenia gravis?
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thymic tumor, thyrotoxicosis, rheumatoid arthritis, lupus erythematosus
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Which disease when treated with a certain drug can present with a myasthenia like syndrome?
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penicillamine for rheumatoid arthritis
resolves with drug discontinuation |
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In whom with myasthenia are corticosteroids given?
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patients who have responded poorly to anticholinesterase drugs and have undergone thymectomy
Treatment must be started in hospital |
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What should be done in rapidly deteriorating patients with myasthenic crisis?
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Plasmapheresis, IVIG
May use prior to surgery and other special circumstances |
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What is best lab test for Lambert Eaton?
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autoantibodies to P/Q subtype of voltage gated calcium channels
highly sensitive, specific |
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What therapy is good for lambert eaton but is not used for myasthenia gravis?
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Guanidine hydrochloride in 3-4 divided doses
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Which medications used in myashtenia gravis have variable response in Lambert Eaton?
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Neostigmine, Pyridiostigmine
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Clinical findings in botulinum toxicity in adults and children?
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Adults - 12-72 hours after ingestion - diplopia, ptosis, facial weakness, dysphagia, nasal speech, difficulty with respiration, weakness appering last in limbs. blurring of vision is classic, dryness of mouth, apralytic ileus, postural hypotension
No sensory deficit. Infants - enteric infection with local prduction of toxin - hypotonia, constipation, progressive weakness, poor suck |
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Which syndrome is mimicked by large doses of aminoglycoside antibiotics?
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Botulinum toxicity
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Treatment for Duchenne dystrophy?
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No definitive therapy but prednisone daily improves muslce strength for up to 3 years
deflazacort is analogue for prednisone and is as effective as prednisone with fewer side effects creatine monohydrate is also beneficial |
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Differences between Duchenne msucluar dystrophy and Becker dystrophy
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average age of onset for becker dystrophy is older and age of death is later (onset - 11, death 42)
cardiac, cognitive impairement do not occur CK levels aer less elevated |
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Features of Emery Dreifuss msuclar dystrophy?
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X linked recessive (Rarer autosomal dominant, recessive forms)
clinical onset in childhood by slow preogression with development of contractures particularily of triceps and biceps in arms, peronei and tibilaris anterior in legs, later spread to girdle muscles cardiac conduction abnormalities, cardiomyopathy CK is mildly increased |
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Inheritance of limb girdle? How does it differ from becker, duchenne dystrophy?
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Autosomal recessive inheritance
disorder begins in late childhood, early adulthood. In contrast to duchenne, becker shoulder and pelvic girdle are equally affected No pseudohypertrophy, less elevation in CK |
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Inheritance and features of facioscapulohumeral dystrophy?
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Autosomal dominant disorder
Onset in adolesence, weakness confined to face, neck, shoulder girdle, foot drop can occur, winged scapulae are common Heart is not involved, serum CK levels are normal or with slight elevation |
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Inheritance and features of distal myopathy?
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autosomal dominant variety presenting after age 40
onset may be earlier and symptoms more severe in homozygotes Small muscles of the hands and feet, wrist extensors, dorsiflexors of foot are affeted slow progressive course |
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Inheritance and features of ocular dystrophy?
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autosomal dominant
onset usually before 30 years ptosis is earliest mainfestation - progressive external ophthalmoplegia subsequently facial weakness is common, subclinical involvement of limb muscles slow progressive course |
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Inheritance, geographic distribution and features of oculopharyngeal dystrophy
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AD
Quebec, southwestern US begins in 3rd to 5th decade ptosis, total external opthalmoplegia, dysphagia, facial weakness , proximal limb weakness mild CK elevation dysphagia is often incapacitating |
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Features of paraspinal dystrophy?
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progressive paraspinal weakness may develop after age of 40
back pain and marked kyphosis ("bent spine syndrome") mildly elevated CK CT with fatty replacement of paraspinal muscles. |
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What are the muscluar dystrophy syndromes?
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Duchenne dystrophy
Becker dystrophy Emery-Dreifuss muscular dystrophy Limb-Girdle dystrophy Facioscapulohumeral dystrophy Distal myopathy Ocular dystrophy Oculopharyngeal dystrophy Paraspinal dystrophy |
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What are the more common congenital myopathies?
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nemaline myopathy - rod shaped bodies in muscle fibers (often also seen in AIDs related myopathy)
central core disease - (association with malignant hypothermia) Myotubular and centronuclear myopathy Mitochondrial myopathies (Kearns-Sayre-Daroff) |
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Describe the mitochondrial myopathies
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Kearns Sayre Daroff - progressive external ophthalmoplegia with pigmentary degeneration of retina, cardiac conduction defects, cerebellar ataxia
MERRF - myoclonic epilepsy, ragged red fiber syndrome MELAS - mitochondrial myopathy, encephalopathy, lactic acidosis, strokelike episodes |
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What is MNGIE and how does it present?
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Mitochondrial neurogastrointestinal encephalopmyopathy
AR disorder affecting mitochondrial function GI dysmotility, skeletal muscle abnormalities |
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What are myotonias generally?
Physical exam maneuver which can sometimes demonstrate myotonia? |
disorders characterized by abnormalities of muscle fiber membrane (sacrolemma) leading ot delay before affected muscles can relax after contraction - apparent muscle stiffness
difficulty relaxing hand after sustained grip or persistent contraction after percussion of belly of muscle |
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How does myotonic dystrophy type 1 typically present?
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manfests typically in third or fourth decade
CTG repeat on 19 myotonia with weakness and wasting of facial, sternomastoid, distal limb muscles Cataracts, frontal balness, testicular atrophy, DM, cardiac abnormalties, intellectual chagnes |
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How does proximal myotonic myopathy present?
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Myotonia, cataracts, proximal weakness
less severe course than DM1 |
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How is myotonia typically treated?
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quinine sulfate, procainamide, or phenytoin
phenytoin is drug of chioce as it is least likely to cause cardiac conduction problems |
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What is Thomsen disease and how does it present?
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Myotonia congenita
Genearlized myotonia without weakness present from birth (sometimes symptoms don't develop until childhood) muscle stiffness enhanced by cold and inactivity and relieved by exercise |
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What are infectious causes of myopathy?
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trichnosis, toxoplasmosis, AIDs
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How does inclusion body myositis typically present?
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insidious onset in those over 50 with painless proximal weakness of lower and then upper extremities
Progressive disease associated with early depression of knee reflexes steroids and immunosuppresives don't work. IVIG is controversial |
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Describe the myopathies caused by HIV infection
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polymyositis - resembles polymositis without AIDs- may respond to corticosteroids
Myopathy associated with type II muscle fiber atrophy Rod-body myopathy - rod shaped bodies and selective loss of thick filaments. Treatment with corticosteroids or plasmapheresis is sometimes helpful mitochondrial myopathy (with biopsy showing ragged red fibers) sometimes occurs in those receiving zidovudine Acute rhabdomyolysis |
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How does paramyotonia congenita present?
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dominantly inherited disorder
weakness and myotonia provoked by cold and worsened by exercise, attacks of hyperkalemic periodic paralysis may occur |
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Describe the alcoholic myopathies?
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Acute necrotizing myopathy- develops over 1-2 days - weakness proximal in distribution, sometimes asymmetric, or focal, moderately or severly elevated CK and myoglobinuria
Recovery in weeks to months with abstience, good nutrition Chronic myopathy - proximal weakness of lower limbs developing insidiously over weeks to months in alcoholic patients. improvement with abstinence in months |
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How does stiff person syndrome present?
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Sporadic, slowly progressive disorder manifested by tightness, stiffness, rigidity of axial and proximal limb muscles with superimposed painful spasms sometimes accompanied by hyperhidrosis and increase in blood pressure
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Which lab findings can help in diagnosis of stiff person syndrome?
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autoantibodies to glutamic acid decarboxylase
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Treatment of stiff person syndrome?
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diazepam
also baclofen, vigabatrin, sodium valproate, gabapentin to relieve symptoms IVIG is sometimes effective in refractory cases |
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What is the eponym for neuromyotonia?
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Isaacs syndrome
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How does neuromyotonia present?
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continuous muscle stiffness, rippling muscle movements (myokymia), delayed relaxation following muscle contraction
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Etiology of neuromyotonia?
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AD inheritance in some
paraneoplastic disorder or in association with other autoimmune diseases or hereditary or sensory neuropathies, following irradiation of the nervous system. |
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Lab finding in acquired neuromyotonia?
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antibodies against voltage gated potassium channels in serum and CSF
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Treatment for neuromyotonia?
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Phenytoin or carbamazepine TID
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What are signs of tetany and causes?
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Hyperexitable state of peripheral nerves (Chvostek sign, Trousseau sign, carpopedal spasm)
hypocalcemia, hypomagnesemia, alkalosis |
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How does hemifacial spasm present?
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repetitive involuntary contractions of some or all of the muscles supplied by one facial nerve. Symptoms commence in orbicularis oculi and spread to cheek and levator anguli oris muscles, breif initial contractions but more sustained with disease pregression
may be provoked by blinking or voluntary activity |
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What commonly causes hemifacial spasm?
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anomalous blood vessel pressing on facial nerve
some claim altered excitability of nerve nucleus in brainstem |
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Treatment for hemifacial spasm?
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carbamazepine or phenytoin
botulinum toxin into affected muscle microvascular decompression |
