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Flashcards in diseases of the motor unit Deck (54):
1

fasciculations are a feature of LMN or UMN

LMN lesion

2

motor functional deficits that define neuromuscular disorders

distribution, weakness, atrophy, cramps

3

sensory functional deficits that define neuromuscular disorders

distribution, parasthesias, dysthesias

4

bulbar involvement may be indicated by what sx?

dysphagia, dysarthria

5

myopathy or neuropathy: presents more commonly with proximal muscle weakness

myopathy

6

distal muscle weakness

neuropathy

7

presents with mixed LMN and UMN lesion signs

ALS

8

ALS affects which portions of SC (2)

1. anterior horn
2. corticospinal tract

9

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

ALS

10

what does ALS typically spare (4)

1. cognition
2. EOM
3. sphincters
4. CSF

11

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

bulbar region+2 spinal regions OR
3 spinal regions

12

20% of ALS patients have what gene mutation

SOD

13

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

riluzole

14

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

mononeuropathy; caused by trauma, entrapment, compression

15

multifocal mononeuropathies; typically caused by_____

mononeuritis multiplex; caused by vasculitis

16

neuropathic pain following nonpainful stimuli

allodynia

17

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

dysesthesia

18

heightened pain sensation provoked by painful stimulus

hyperalgesia

19

irritating spontaneous sensation

parasthesia

20

stocking glove neuropathy with distal weakness

peripheral neuropathy

21

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

charcot-marie-tooth disease

22

what is the most common inherited neuro disorder

charcot-marie-tooth disease

23

another name for charcot-marie-tooth disease

hereditary motor sensory neuropathy

24

type ____HSMN is demyelinating with ____NCV

type 1; slowed NCV

25

type _____HSMN is axonal with _____NCV

type 2; normal or mildly slowed NCV

26

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

acute inflammatory demyelinating neuropathy (guillain barre)

27

opthalmoplegia + ataxia + loss of DTR

fisher syndrome

28

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

guillain barre

29

gold standard tx for guillain barre

plasmapharesis

30

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

IVIG

31

most common type of diabetic neuropathy

distal symmetric polyneuropathy

32

EMG can distinguish what?

b/w axonal and demyelinating neuropathy

33

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

myasthenia gravis

34

___extremities are more commonly affected by muscle weakness in myasthenia gravis

upper

35

respiratory failure secondary to intercostal and diagphragmatic weakness

myasthenic crisis

36

myasthenia gravis pathophys is due to anti_____Ab that block _____synaptic receptros

anti-ACh Ab that block post synaptic receptors

37

pharmacologic dx of myasthenia gravis

edrophonium test blocks AChE

38

T or F: Ab titers indicate disease severity of MG

FALSE

39

what can be used for sx tx of MG?

cholinesterase inhibs (mestinon)

40

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

myasthenic crisis

41

the hallmark of myopathy is _____

weakness

42

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

myopathy

43

what marker is often elevated in myopathy

CK

44

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

polymyositis

45

biopsy changes expected with polymyositis

Ag specific cytotoxic t cells, direct muscle fiber damage

46

subacute proximal weakness with heliotrope rash, Grotton's patches

dermatomyositis

47

Ab against endothelial cells dx what?

dermatomyositis

48

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

inclusion body myositis

49

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

>30 mg QD for >2 months

50

any muscle complaint related to statins

statin myopathy

51

muscle complaints w/o CK elevation

myalgia

52

muscle sx w/ CK elevation

myositis

53

CK elevation >10x normal with incr Cr

rhabdomyolysis

54

asymmetric, variable location of motor sx, atrophy, fasciculations

motor neuron disease