diseases of the motor unit Flashcards

1
Q

fasciculations are a feature of LMN or UMN

A

LMN lesion

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2
Q

motor functional deficits that define neuromuscular disorders

A

distribution, weakness, atrophy, cramps

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3
Q

sensory functional deficits that define neuromuscular disorders

A

distribution, parasthesias, dysthesias

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4
Q

bulbar involvement may be indicated by what sx?

A

dysphagia, dysarthria

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5
Q

myopathy or neuropathy: presents more commonly with proximal muscle weakness

A

myopathy

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6
Q

distal muscle weakness

A

neuropathy

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7
Q

presents with mixed LMN and UMN lesion signs

A

ALS

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8
Q

ALS affects which portions of SC (2)

A
  1. anterior horn

2. corticospinal tract

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9
Q

weakness, atrophy, fasciculations, spasticity, hyperreflexia; sparing sensory fxn

A

ALS

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10
Q

what does ALS typically spare (4)

A
  1. cognition
  2. EOM
  3. sphincters
  4. CSF
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11
Q

dx of ALS: UMN + LMN lesion signs in _____region + ____spinal regions OR ____spinal regions

A

bulbar region+2 spinal regions OR

3 spinal regions

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12
Q

20% of ALS patients have what gene mutation

A

SOD

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13
Q

glutamate antag, with 2-3 month prolonged survival in ALS

A

riluzole

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14
Q

individual disorder of single nerve; typically cause by ____(3)

A

mononeuropathy; caused by trauma, entrapment, compression

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15
Q

multifocal mononeuropathies; typically caused by_____

A

mononeuritis multiplex; caused by vasculitis

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16
Q

neuropathic pain following nonpainful stimuli

A

allodynia

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17
Q

unpleasant sensation that is either spontaneous or evoked by pressure/movement/touch

A

dysesthesia

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18
Q

heightened pain sensation provoked by painful stimulus

A

hyperalgesia

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19
Q

irritating spontaneous sensation

A

parasthesia

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20
Q

stocking glove neuropathy with distal weakness

A

peripheral neuropathy

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21
Q

pes cavus, distal muscle weakness/wasting in legs, difficulty walking

A

charcot-marie-tooth disease

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22
Q

what is the most common inherited neuro disorder

A

charcot-marie-tooth disease

23
Q

another name for charcot-marie-tooth disease

A

hereditary motor sensory neuropathy

24
Q

type ____HSMN is demyelinating with ____NCV

A

type 1; slowed NCV

25
Q

type _____HSMN is axonal with _____NCV

A

type 2; normal or mildly slowed NCV

26
Q

ascending paralysis comes on over days-weeks; spared sensory; loss of DTR; high alb with nml WBC

A

acute inflammatory demyelinating neuropathy (guillain barre)

27
Q

opthalmoplegia + ataxia + loss of DTR

A

fisher syndrome

28
Q

most common cause of acute, nontraumatic generalized paralysis in young adults

A

guillain barre

29
Q

gold standard tx for guillain barre

A

plasmapharesis

30
Q

what else can be used to tx guillain barre other than plasma exchange

A

IVIG

31
Q

most common type of diabetic neuropathy

A

distal symmetric polyneuropathy

32
Q

EMG can distinguish what?

A

b/w axonal and demyelinating neuropathy

33
Q

fluctuating weakness, incr with exertion and improved with rest; diplopia, ptosis

A

myasthenia gravis

34
Q

___extremities are more commonly affected by muscle weakness in myasthenia gravis

A

upper

35
Q

respiratory failure secondary to intercostal and diagphragmatic weakness

A

myasthenic crisis

36
Q

myasthenia gravis pathophys is due to anti_____Ab that block _____synaptic receptros

A

anti-ACh Ab that block post synaptic receptors

37
Q

pharmacologic dx of myasthenia gravis

A

edrophonium test blocks AChE

38
Q

T or F: Ab titers indicate disease severity of MG

A

FALSE

39
Q

what can be used for sx tx of MG?

A

cholinesterase inhibs (mestinon)

40
Q

besides tx of guillain barre, plasmapharesis and IVIG can be used to tx what?

A

myasthenic crisis

41
Q

the hallmark of myopathy is _____

A

weakness

42
Q

weakness that is symmetric and proximal, neck flexor weakness, waddling gait

A

myopathy

43
Q

what marker is often elevated in myopathy

A

CK

44
Q

painless subacute proximal weakness with dysphagia, neck weakness that spares eyes, face

A

polymyositis

45
Q

biopsy changes expected with polymyositis

A

Ag specific cytotoxic t cells, direct muscle fiber damage

46
Q

subacute proximal weakness with heliotrope rash, Grotton’s patches

A

dermatomyositis

47
Q

Ab against endothelial cells dx what?

A

dermatomyositis

48
Q

weakness over years, quadriceps weakness, no respose to immunosuppressive agents

A

inclusion body myositis

49
Q

steroid myopathy can present when on doses prednisone >____ for >_____montsh

A

> 30 mg QD for >2 months

50
Q

any muscle complaint related to statins

A

statin myopathy

51
Q

muscle complaints w/o CK elevation

A

myalgia

52
Q

muscle sx w/ CK elevation

A

myositis

53
Q

CK elevation >10x normal with incr Cr

A

rhabdomyolysis

54
Q

asymmetric, variable location of motor sx, atrophy, fasciculations

A

motor neuron disease