Neuromuscular Disorders Flashcards

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
Q

what is the most common cause of plexopathy?

A

trauma

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26
Q

polyneuropathies are _____ dependent

A

length

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27
Q

plexopathy sx

A

severe pain then weakness

28
Q

what is meralgia parasthetica?

A

lateral femoral cutaneous compressed at inguinal ligament
sensory loss ONLY

29
Q

carpal tunnel is an example of

A

mononeuropathy

30
Q

most myelinated fibers are _____ diameter fibers

A

large

31
Q

what is a strong characteristic of demylination?

A

weakness w/o atrophy

32
Q

axons regrow _____ per year

A

2-3 mm

33
Q

what is the most common axonal polyneuropathy? 2nd?

A

most - diabetes
2nd - alcohol

34
Q

T/F: EMG can be normal for small fiber polyneuropthies

A

T

35
Q

what sense composes small fiber neurons?

A

sensory - pain and temp
autonomic

36
Q

small fiber neuropathy sx

A

reduced temp discrimination
allodynia
burning, neuropathic pain
LE discoloration, reduced sweating, & hair loss
autonomic dysfunction

37
Q

what type of nerves are large fibers?

A

motor
sensory - balance & proprioception

38
Q

what is the 1st sign of autonomic neuropathy?

A

erectile dysfunction

39
Q

sx of autonomic neuropathy

A

orthostatic hypotension
arrythmias
severe constipation, urinary retention
erectile dysfunction
abnormal sweating
early satiety, lightheaded with meals

40
Q

what are the rapidly progressive inflammatory demyelinating polyneuropathies?

A

AIDP
Guillain-Barre

41
Q

what are the slowly progressive inflammatory demyelinating polyneuropathies?

A

CIDP
weakness progressing > 2 months

42
Q

how does acute inflammatory demyelinating polyneuropathy progress?

A
  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
Q

where is the most common area of weakness in acute inflammatory demyelinating polyneuropathy?

A

face

44
Q

what labs are elevated with acute inflammatory demyelinating polyneuropathy?

A

protein

45
Q

T/F: steroids are beneficial for Guillain Barre

A

F

46
Q

Guillain Barre is a demyelinating ____ illness

A

monophasic

47
Q

which fibers are lost with chronic inflammatory demyelinating polyneuropathy?

A

large motor

48
Q

sx of chronic inflammatory demyelinating polyneuropathy

A

motor loss
symmetric weakness proximal AND distal
HYPO and Areflexic
sensory loss in LE (not disabling)

49
Q

T/F: diabetic neuropathy should NOT cause weakness

A

T

50
Q

are UEs or LEs affected first with diabetic neuropathy?

A

LE

51
Q

T/F: the pain and numbness can be treated for diabetic neuropathy

A

F: just pain

52
Q

presentation of diabetic lumbosacral radiculoplexus neuropathy (DLRPN) aka diabetic amytrophy

A

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
Q

Charcot-Marie-Tooth Disease clinical manifestations

A

distal weakness and atrophy
length dependent sensory loss
foot deformities
absent or decreased reflexes
chronic slowly progressive

54
Q

when is Charcot-Marie-Tooth Disease most commonly onset?

A

children

55
Q

Lambert Eaton is a ___synaptic disorder

A

PRE

56
Q

Myasthenia gravis is a ____synaptic disorder

A

POST

57
Q

diff dx/hallmark of MG

A

fluctuating, fatigable weakness especially of bilateral eyes that has a quick improvement with rest

58
Q

myopathies usually have ___ weakness

A

proximal

59
Q

polymyositis and dermatomyositis presentation

A

subacute/chronic onset of prox weakness
variable cardio and pulm involvement
d/t malignancy, autoimmune disorders, HIV

60
Q

1st line of treat for dermatomyositis

A

steroids

61
Q

2nd line of treatment for dermatomyositis

A

mild chemo

62
Q

3rd line of treat dermatomyositis

A

usually related to cancer - chemo

63
Q

how does weakness present in inclusion body myositis?

A

proximal leg and distal arm

64
Q

T/F: steroid help with inclusion body myositis

A

F: none

65
Q

what is the most common myopathy in adults?

A

myotonia

66
Q

which muscular dystrophy is common in children?

A

Duchene & Becker’s