NMJ Disorders Flashcards

1
Q

Safety factor at normal NMJs

A

EPP amplitudes dec during repetitive muscle contraction so less amount of ACh is released, but it’s still above threshold and gives adequate muscle contraction

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2
Q

Autoimmune disorder of post-synaptic NMJ

A

Myasthenia gravis

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3
Q

2 NMJ disorders that affect the pre-synaptic NMJ

A

Botulism and Lambert Eaton

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4
Q

target of Ab for myasthenia vs lambert eaton

A

myasthenia - AChR

lambert eaton - voltage gated Ca channel

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5
Q

myasthenia under electron microscopy

A

loss of architecture/folding of post-synaptic NMJ

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6
Q

initial sxs of myasthenia

A

ptosis, diplopia, dysarthria, dysphagia

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7
Q

is myasthenia symmetrical?

A

not always

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8
Q

neonatal myasthenia

A

healthy newborns of myasthenic moms have sxs until maternal antibodies wash out of their system

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9
Q

myasthenic crisis

A

profound weakness that may cause quadriplegia w/ patient unable to speak/swallow/breathe

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10
Q

triggers of myasthenic crisis

A

infection or systemic illness

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11
Q

diff dx for myasthenic crisis

A

extensive brain infarct, guillan barre, acute paralysis from spinal cord lesion (via inflamm or tumor)

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12
Q

how are sensation and reflexes affected w/ myasthenic crisis?

A

they’re normal

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13
Q

most specific diagnostic test for myasthenia

A

serum AChR Ab

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14
Q

2 Ab for myasthenia

A
AChR Ab
MuSK Ab (tyrosine kinase)
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15
Q

high doses of AChE inhibitors can give

A
cholinergic crisis (weakness, sweatiness, salivation, diarrhea, urination)
"wet sxs"
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16
Q

other tx for myasthenia besides AChE inhibitors

A

thymectomy, immunosuppressants, corticosteroids, IVIG, plasmapheresis

17
Q

weakness of proximal muscles of shoulders, hips, trunk

A

myopathy or lambert eaton

18
Q

autonomic sxs of lambert eaton

A

dry mouth, orthostatic hypotension, erectile dysfunction

19
Q

lambert eaton associated with

A

sm cell ca of lung

20
Q

tx to dec fatigue and improve strength for lambert eaton via inc ACh release

A

guanidine or 3,4-diaminopyridine