Neuromuscular Disorders Flashcards

(66 cards)

1
Q

myelin is composed of ___ cells in the PNS

A

Schwann

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2
Q

what do small fibers sense?

A

pain and temperature

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3
Q

what do large fibers sense?

A

light touch and vibration

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4
Q

how do the nerves exit the SC?

A

cervical: above vertebral level
T-S: below

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5
Q

what is the hallmark of ALS disease?

A

UMN & LMN sx
motor involvement ONLY

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6
Q

LMN signs in ALS

A

weakness
atrophy
fasciculations all over body

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7
Q

UMN signs in ALS

A

pathologic spread of reflexes
clonus
spasticity

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8
Q

T/F: there is no sensory loss or cognitive decline with ALS

A

T

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9
Q

how effective are ALS meds?

A

only prolong life for about 3 months

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10
Q

meds for ALS

A

anyone: riluzole & edaravone
genetic: tofersen

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11
Q

which intervention prolongs life for ALS pts better?

A

BIPAP

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12
Q

____ physical activity increases risk for ALS patients

A

intense

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13
Q

what is the most common motor neuron disease?

A

ALS

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14
Q

West Nile sx

A

weakness in B legs
fever, drowsy
stiff neck & HA
absent reflexes in LE
july-october

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15
Q

how to differentiate neuron injury d/t acute flaccid paralysis vs. Guillain-Barré

A

AFP: B/B involvement and no image changes

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16
Q

sx of neuro involvement d/t West Nile

A

aseptic meningitis
meningoencephalitis
acute flaccid paralysis
B/B involvement

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17
Q

post-polio syndrome diagnostic criteria

A
  1. hx of paralytic poliomyelitis
  2. period of partial or complete RECOVERY followed by interval of stable function
  3. gradual or sudden onset of gradual or sudden progressive and persistent MUSCLE WEAKNESS/FATIGUE
  4. sx for > 1 year
  5. exclude other causes
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18
Q

post-polio syndrome causes _____ neuron syndrome

A

LMN

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19
Q

what is the most common level for radiculopathy?

A

L5

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20
Q

what is the most common level for nerve injury?

A

C7

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21
Q

sx of radiculopathy

A

pain, numbness and tingling dermatomal
weakness w/ corresponding myotome
reduced reflexes

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22
Q

T/F: 95% of cervical radiculopathies d/t herniated disc improve w/o surgical intervention

A

T

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23
Q

pain difference b/w radiculopathy and LMN lesion?

A

radic = shooting pain

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24
Q

ABCDs of radiculopathy vs. LMN lesion

A

A - weakness
B - radicular pain in dermatomal dist
C - muscle atrophy
D - hyporeflexia

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25
what is the most common cause of plexopathy?
trauma
26
polyneuropathies are _____ dependent
length
27
plexopathy sx
severe pain then weakness
28
what is meralgia parasthetica?
lateral femoral cutaneous compressed at inguinal ligament sensory loss ONLY
29
carpal tunnel is an example of
mononeuropathy
30
most myelinated fibers are _____ diameter fibers
large
31
what is a strong characteristic of demylination?
weakness w/o atrophy
32
axons regrow _____ per year
2-3 mm
33
what is the most common axonal polyneuropathy? 2nd?
most - diabetes 2nd - alcohol
34
T/F: EMG can be normal for small fiber polyneuropthies
T
35
what sense composes small fiber neurons?
sensory - pain and temp autonomic
36
small fiber neuropathy sx
reduced temp discrimination allodynia burning, neuropathic pain LE discoloration, reduced sweating, & hair loss autonomic dysfunction
37
what type of nerves are large fibers?
motor sensory - balance & proprioception
38
what is the 1st sign of autonomic neuropathy?
erectile dysfunction
39
sx of autonomic neuropathy
orthostatic hypotension arrythmias severe constipation, urinary retention erectile dysfunction abnormal sweating early satiety, lightheaded with meals
40
what are the rapidly progressive inflammatory demyelinating polyneuropathies?
AIDP Guillain-Barre
41
what are the slowly progressive inflammatory demyelinating polyneuropathies?
CIDP weakness progressing > 2 months
42
how does acute inflammatory demyelinating polyneuropathy progress?
1. tingling, paresthesias w/o sensory loss 2. severe radicular pain 3. weakness (starts in legs --> up) gets worse over 4 weeks then plateus no B/B involvement
43
where is the most common area of weakness in acute inflammatory demyelinating polyneuropathy?
face
44
what labs are elevated with acute inflammatory demyelinating polyneuropathy?
protein
45
T/F: steroids are beneficial for Guillain Barre
F
46
Guillain Barre is a demyelinating ____ illness
monophasic
47
which fibers are lost with chronic inflammatory demyelinating polyneuropathy?
large motor
48
sx of chronic inflammatory demyelinating polyneuropathy
motor loss symmetric weakness proximal AND distal HYPO and Areflexic sensory loss in LE (not disabling)
49
T/F: diabetic neuropathy should NOT cause weakness
T
50
are UEs or LEs affected first with diabetic neuropathy?
LE
51
T/F: the pain and numbness can be treated for diabetic neuropathy
F: just pain
52
presentation of diabetic lumbosacral radiculoplexus neuropathy (DLRPN) aka diabetic amytrophy
begins abrupt sharp or aching pain asymmetrically in hip and thigh ---> leg and foot pain --> weakness thigh and knee --> weakness progresses distally often becomes bilateral
53
Charcot-Marie-Tooth Disease clinical manifestations
distal weakness and atrophy length dependent sensory loss foot deformities absent or decreased reflexes chronic slowly progressive
54
when is Charcot-Marie-Tooth Disease most commonly onset?
children
55
Lambert Eaton is a ___synaptic disorder
PRE
56
Myasthenia gravis is a ____synaptic disorder
POST
57
diff dx/hallmark of MG
fluctuating, fatigable weakness especially of bilateral eyes that has a quick improvement with rest
58
myopathies usually have ___ weakness
proximal
59
polymyositis and dermatomyositis presentation
subacute/chronic onset of prox weakness variable cardio and pulm involvement d/t malignancy, autoimmune disorders, HIV
60
1st line of treat for dermatomyositis
steroids
61
2nd line of treatment for dermatomyositis
mild chemo
62
3rd line of treat dermatomyositis
usually related to cancer - chemo
63
how does weakness present in inclusion body myositis?
proximal leg and distal arm
64
T/F: steroid help with inclusion body myositis
F: none
65
what is the most common myopathy in adults?
myotonia
66
which muscular dystrophy is common in children?
Duchene & Becker's