Motor Neuron Diseases Flashcards

1
Q

associated symptoms of motor neuron lesions

A

table 3

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2
Q

t/f on PE look for both umn and lmn lesion signs aside from weakness (atrophy, fasciculations, abnormal reflexes)

A

true

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3
Q

common mnd mimics

A

myasthenia gravis
neurosyphilis
post-polio syndrome
thyroroxicosis

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4
Q

lesions in als

A

umn and lmn

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5
Q

lesions in pma

A

purely lmn

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6
Q

lesions in pls

A

purely umn

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7
Q

lesions in pbp

A

umn and lmn

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8
Q

familial mutation in als

A

superoxide dismutase type 1 or sod 1

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9
Q

t/f death occurs in als after 6 years of diagnosis

A

false, 2-5 years

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10
Q

pathophysiology of als

A

unknown
motor neuron susceptibility factors can lead no mni
glutamate toxicity can lead to cell death

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11
Q

el esocorial federation for diagnosis of als

A

signs of lmn and umn degeneration

progressive spread of signs with absence of ecg and imaging evidence

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12
Q

revised el esocorial criteria

A

table 5

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13
Q

awaji shima categories

A

table 6

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14
Q

mri findings in als

A

used to exclude mnd mimics

hyperintensities or atrophy in precentral gyrus

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15
Q

what is ncv

A

examines sensory and motor nerve action potentials

can distinguish axonal and demyelinating lesions

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16
Q

results of axonal vs demyelinating lesions

A

axonal: abnormal or reduced amplitude of sensory or motor aps
demyelinating: abnormal latencies and/or conduction velocities

17
Q

ncv results of patient with als

A

sensory: normal
motor: normal conduction velocity, reduced amplitude

18
Q

what is emg

A

examines motor unit action potentials

discriminates neuropathic and myopathic source

19
Q

emg results in als

A

spontaneous activities (fasciculation, fibrillation, sharp waves)
motor unit aps are large
recruitment pattern is reduced

20
Q

pls vs pma

21
Q

pathophysiology of spinal muscular atrophy

A

autosomal recessive disorder caused by mutations in spinal motor neuron gene

22
Q

clinical manifestations of sma

A

weakness of varied severity depending on type and age of onset
proximal muscles more affected

23
Q

types of sma

A

1: werdnig-hoffman
2: dubowitz
3: wohlfart-kugelberg-welander
4: after 50

24
Q

pathophysiology of kennedy’s disease

A

adult onset sma

x linked recessive

25
clinical features of kennedy's disease
slowly progressive bulbar weakness and atrophy proximal > distal eom sparing gynecomastia and testicular atrophy
26
pathophysiology of hirayama disease
focal ischemic changes in anterior horn cells of lower cervical cord
27
clinical features of hirayama disease
progressive wasting of muscles in hand and forearm brachioradialis sparing progresses then stabilizes
28
pathophysio of acute poliomyelitis
due to poliovirus going through oral route to cns - spinal cord (lumbosacral > cervical > thoracic) - nucleus ambiguus, cn 5, 7, 12
29
clinical features of acture poliomyelitis
minor: nonspecific major: headache, fever, vomiting, malaise, neck and back stiffness, asymmetric weakness, hypo/areflexia
30
clinical features of post-poliomyelitis syndrome
new onset 20-30 years after infection | fatigue, joint pains, muscle pain, weakness, atrophy
31
associated cancer with paraneoplastic motor neuron disorder
subacute motor neuronopathy: lymphoma | paraneoplastic encephalomyelitis: sclc and (+) anti-hu antibodies
32
anti-glutamate drug that can improve als prognosis
riluzole
33
new drug to treat als
edavarone
34
signs of poor prognosis in als
``` older short interval between symptoms onset and diagnosis weight loss reduced pulmo function muscle weakness bulbar onset of disease ```