diseases of the motor unit

Question 1

fasciculations are a feature of LMN or UMN

Answer 1

LMN lesion

Question 2

motor functional deficits that define neuromuscular disorders

Answer 2

distribution, weakness, atrophy, cramps

Question 3

sensory functional deficits that define neuromuscular disorders

Answer 3

distribution, parasthesias, dysthesias

Question 4

bulbar involvement may be indicated by what sx?

Answer 4

dysphagia, dysarthria

Question 5

myopathy or neuropathy: presents more commonly with proximal muscle weakness

Answer 5

myopathy

Question 6

distal muscle weakness

Answer 6

neuropathy

Question 7

presents with mixed LMN and UMN lesion signs

Answer 7

ALS

Question 8

ALS affects which portions of SC (2)

Answer 8

1. anterior horn

2. corticospinal tract

Question 9

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

Answer 9

ALS

Question 10

what does ALS typically spare (4)

Answer 10

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

Question 11

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

Answer 11

bulbar region+2 spinal regions OR

3 spinal regions

Question 12

20% of ALS patients have what gene mutation

Answer 12

SOD

Question 13

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

Answer 13

riluzole

Question 14

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

Answer 14

mononeuropathy; caused by trauma, entrapment, compression

Question 15

multifocal mononeuropathies; typically caused by_____

Answer 15

mononeuritis multiplex; caused by vasculitis

Question 16

neuropathic pain following nonpainful stimuli

Answer 16

allodynia

Question 17

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

Answer 17

dysesthesia

Question 18

heightened pain sensation provoked by painful stimulus

Answer 18

hyperalgesia

Question 19

irritating spontaneous sensation

Answer 19

parasthesia

Question 20

stocking glove neuropathy with distal weakness

Answer 20

peripheral neuropathy

Question 21

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

Answer 21

charcot-marie-tooth disease

Question 22

what is the most common inherited neuro disorder

Answer 22

charcot-marie-tooth disease

Question 23

another name for charcot-marie-tooth disease

Answer 23

hereditary motor sensory neuropathy

Question 24

type ____HSMN is demyelinating with ____NCV

Answer 24

type 1; slowed NCV

Question 25

type _____HSMN is axonal with _____NCV

Answer 25

type 2; normal or mildly slowed NCV

Question 26

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

Answer 26

acute inflammatory demyelinating neuropathy (guillain barre)

Question 27

opthalmoplegia + ataxia + loss of DTR

Answer 27

fisher syndrome

Question 28

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

Answer 28

guillain barre

Question 29

gold standard tx for guillain barre

Answer 29

plasmapharesis

Question 30

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

Answer 30

IVIG

Question 31

most common type of diabetic neuropathy

Answer 31

distal symmetric polyneuropathy

Question 32

EMG can distinguish what?

Answer 32

b/w axonal and demyelinating neuropathy

Question 33

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

Answer 33

myasthenia gravis

Question 34

___extremities are more commonly affected by muscle weakness in myasthenia gravis

Answer 34

upper

Question 35

respiratory failure secondary to intercostal and diagphragmatic weakness

Answer 35

myasthenic crisis

Question 36

myasthenia gravis pathophys is due to anti_____Ab that block _____synaptic receptros

Answer 36

anti-ACh Ab that block post synaptic receptors

Question 37

pharmacologic dx of myasthenia gravis

Answer 37

edrophonium test blocks AChE

Question 38

T or F: Ab titers indicate disease severity of MG

Answer 38

FALSE

Question 39

what can be used for sx tx of MG?

Answer 39

cholinesterase inhibs (mestinon)

Question 40

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

Answer 40

myasthenic crisis

Question 41

the hallmark of myopathy is _____

Answer 41

weakness

Question 42

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

Answer 42

myopathy

Question 43

what marker is often elevated in myopathy

Answer 43

CK

Question 44

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

Answer 44

polymyositis

Question 45

biopsy changes expected with polymyositis

Answer 45

Ag specific cytotoxic t cells, direct muscle fiber damage

Question 46

subacute proximal weakness with heliotrope rash, Grotton's patches

Answer 46

dermatomyositis

Question 47

Ab against endothelial cells dx what?

Answer 47

dermatomyositis

Question 48

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

Answer 48

inclusion body myositis

Question 49

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

Answer 49

>30 mg QD for >2 months

Question 50

any muscle complaint related to statins

Answer 50

statin myopathy

Question 51

muscle complaints w/o CK elevation

Answer 51

myalgia

Question 52

muscle sx w/ CK elevation

Answer 52

myositis

Question 53

CK elevation >10x normal with incr Cr

Answer 53

rhabdomyolysis

Question 54

asymmetric, variable location of motor sx, atrophy, fasciculations

Answer 54

motor neuron disease