Neuropathy, Myopathy, & Motor Neuron Dz Flashcards

1
Q

single major named nerve has deficits due to trauma/compression

A

mononeuropathy

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2
Q

localization of mononeuropathy via

A

EMG

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3
Q

disorder of multiple nerves, sxs often symmetrical, sensation affected early, dec reflexes early on

A

polyneuropathy or peripheral neuropathy

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4
Q

why are distal regions affected first for peripheral neuropathy

A

longest nerves affected first b/c metabolic maintenance and axoplasmic flow are more susceptible to neurotoxic factors

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5
Q

spontaneous tingling

“pins and needles”

A

paresthesia

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6
Q

unpleasant sensation from non-noxious stimuli

A

dysesthesia

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7
Q

sxs of polyneuropathy or peripheral neuropathy

A

sxs often symmetrical, sensation affected early, dec reflexes early on, weakness/sensation affected distally first

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8
Q

2 pathological processes in neuropathy

A

demyelination

axonal degeneration

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9
Q

mononeuropathy from focal compression due to

A

demyelination
ex: carpal tunnel
(axonal degen is secondary process)

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10
Q

2 ex of demyelination

A
guillan barre (polyneuropathy)
carpal tunnel (mononeuropathy)
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11
Q

mechanism of axonal loss

A

Wallerian degen - axons and myelin degenerate distal to where nerve was injured

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12
Q

support scaffolding that guides regrowing of axons

A

perineurium

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13
Q

lack of perineurium leads to

A

resprouting axons piling up in a bulbous neuroma (often painful)

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14
Q

primary pathology for polyneuropathies due to toximetabolic cause

A

axonal degen (demyelination is secondary process)

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15
Q

nerve at wrist, elbow, and fibular head

A

wrist - median n
elbow - ulnar n
fibular head - common peroneal n

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16
Q

tests electrical activity and function of nerves and muscles

A

EMG

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17
Q

if EMG suggests chronic polyneuropathy due to inflamm, immune, or vasculitis - confirm dx via

A

sural (sensory n) biopsy

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18
Q

biopsy of sural nerve results in

A

permanent numbness along lateral foot

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19
Q

treatable causes of polyneuropathy - screen for

A
DM
liver/kidney probs
Vit B12 deficiency
hypothyroidism
anemia/leukemia (CBC)
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20
Q

MOA of topical capsaicin

A

substance P depleter

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21
Q

oral meds for neuropathic pain

A

anticonvulsants (gabapentin, pregabalin, carbamazepine) or antidepressants (amitriptyline or duloxetine)

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22
Q

immune system targets peripheral nerve myelin and gives ascending, areflexic paralysis

A

guillan barre (after viral illness, surgery, or trauma)

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23
Q

guillan barre weakness plateaus when

A

after 3-4 wks

24
Q

guillan barre EMG

A

shows asymmetrical demyelination in proximal and distal segments of diff nerves

25
Q

guillan barre CSF

A

may have elevated protein, few wbcs (if any), no signs of infection

26
Q

faster recovery for guillan barre if you administer

A

plasmapheresis (removes circulating ab) or IVIG (gives ab that counteract the autoimmune process)

27
Q

myopathy sxs

A

proximal weakness, nl sensation, late loss of reflexes (after atrophy)

28
Q

rapid weakness from viral induced breakdown of musc fibers seen with

A

influenza

29
Q

meds that can give weakness

A

statins

corticosteroids

30
Q

endocrine dz that can give myopathy

A

cushing’s

hypothyroid

31
Q

dx of myopathy

A

measure creatine kinase (CK)
EMG
muscle biopsy

32
Q

inflammation of multiple muscles, typically autoimmune in US

A

polymyositis

33
Q

myositis w/ periorbital and knuckle rash

A

dermatomyositis

34
Q

muscle biopsy of myositis

A

inflamm cell infiltrates admist necrotic and regenerating musc fibers

35
Q

hereditary myopathies of variable progression/severity

A

musc dystrophies

36
Q

death for duchenne’s caused by

A

weak respiratory musc or cardiomyopathy

37
Q

excessive trinuc repeats on chromosome 19 giving abnl protein kinase

A

myotonic dystrophy type 1, autosomal dominant

38
Q

sxs of myotonic dystrophy

A

weakness of distal limbs, neck, and face, weak jaw closure (often hangs open)

39
Q

impaired relaxation of muscle after volitional contraction

A

myotonia

40
Q

exam step to elicit myotonia

A

percussion of thenar musc w/ reflex hammer

41
Q

other sxs of myotonic dystrophy not neuro related

A

cataracts, frontal baldness, infertility, cardiac arrhythmias

42
Q

tracts for UMN

A

CST - anterior horn cells

corticobulbar tract - CN (LMNs)

43
Q

dz that only involves anterior horn cells, often hereditary

A

SMA

44
Q

SMA sxs

A

LMN signs - weakness, atrophy, fasciculations, hyporeflexia

45
Q

infantile onset SMA

A

Werdnig Hoffman

46
Q

slowly progressive atrophy of one limb or restricted segments of limbs, normal lifespan

A

benign focal amyotrophy

47
Q

familial degen of CST in lateral columns of spinal cord not due to structural/metabolic issues

A

primary lateral sclerosis (weakness + UMN signs)

48
Q

dz where only corticobulbar tract is involved

A

psuedobulbar palsy (facial weakness, dysphagia, hoarseness, impaired chewing, dysarthria) w/ inc jaw jerk

49
Q

cause of pseudobulbar palsy

A

MS lesions, trauma, tumors, b/l mutl cerebral infarcts

50
Q

focal weakness/atrophy in limb (“drop”) that spreads and becomes b/l, male 40-70 y/o

A

ALS

51
Q

ALS that starts w/ dysarthria, hoarseness, or impaired swallowing

A

bulbar ALS

52
Q

sxs of ALS

A

widespread fasciculations, spasticity, hyper-reflexia, atrophy, weakness

53
Q

spared w/ ALS

A

extraocular musc

bladder/bowel fxn

54
Q

familial ALS cause

A

defective superoxide dismutase…free radicals destroy motor neurons

55
Q

tx for ALS

A

no cure, riluzole helps

56
Q

riluzole MOA

A

opposes excitotoxic effect of glutamate at NMDA receptors