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