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Neurology > Disorders of the Neuromuscular Junction > Flashcards

Disorders of the Neuromuscular Junction Flashcards

1
Q

autoimmune disorder that is characterized as muscle weakness due to dysfunction of the neuromuscular junction

A

myasthenia gravis

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2
Q

the most common disorder of neuromuscular transmission

A

myasthenia gravis

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3
Q

myasthenia gravis in which autoantibodies attack the acetylcholine receptor, resulting in reduction of the number of receptors over time

A

seropositive

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4
Q

myasthenia gravis in which the patient has no detectable levels of autoantibodies against acetylcholine receptors

A

seronegative

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5
Q

what antibodies may be present in patients with seronegative myasthenia gravis?

A

antibodies directed against MuSK

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6
Q

presents at birth as weakness due to the mother with myasthenia gravis who passes antibodies through the placenta

A

congenital myasthenia

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7
Q

what is the cardinal feature of myasthenia gravis?

A

fluctuating skeletal muscle weakness

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8
Q

when does the skeletal muscle weakness get worse or improve?

A

worse later in day or with exercise

improves with rest

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9
Q

what are the 2 clinical forms of weakness in myasthenia gravis?

A

ocular
generalized

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10
Q

what is the presentation called when a patient cannot pull facial muscles into a complete smile?

A

myasthenic sneer

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11
Q

what is the presentation called when myasthenia gravis affects the neck muscles?

A

dropped head syndrome

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12
Q

what does myasthenia gravis of the respiratory muscles cause? and what could cause this (4)?

A

myasthenic crisis

surgery
infection
medications
tapering immunosuppression

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13
Q

what will NOT change in myasthenia gravis? (2)

A

sensation
reflexes

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14
Q

what is the diagnostic criteria for myasthenia gravis? (2)

A

fatigability/weakness of muscle + improvement with anticholinesterase drugs

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15
Q

what diagnostic can test ptosis in myasthenia gravis?

A

ice pack test will improve ptosis

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16
Q

what are the 3 serologic tests for myasthenia gravis?

A

acetylcholine receptor antibodies
MuSK antibodies
autoimmune thyroid labs

17
Q

why would we do a chest CT in myasthenia gravis?

A

to look for thymoma

18
Q

what are 2 electrophysiologic studies for myasthenia gravis?

A

repetitive nerve stimulation studies
single-fiber electromyography (EMG)

19
Q

what is the 1st line treatment for myasthenia gravis in a stable patient?

A

oral anticholinesterase (pyridostigmine)

20
Q

what should we watch for when using pyridostigmine?

A

cholinergic crisis

21
Q

what is the 2nd line treatment for myasthenia gravis in a stable patient? (2)

A

chronic immunosuppressive agents:

azathioprine
oral glucocorticoid

22
Q

what are 2 options for treatment of myasthenia gravis in patient who is in potential crisis?

A

plasmapheresis
IVIG

23
Q

what are the 3 uses for plasmapheresis and IVIG?

A

myasthenic crisis
preop before thymectomy
bridge to slower acting immunotherapies

24
Q

what can be done for a patient with myasthenia gravis in a case of thymoma or nonthymomatous autoimmunity?

A

thymectomy

25
Q

what must be done before performing a thymectomy on a patient?

A

control weakness first

26
Q

what is the clinical course of myasthenia gravis? (2)

A

symptom-free periods are lost
progression peaks withing few years of onset

27
Q

myasthenic syndrome associated with small cell carcinoma characterized by weakness of proximal limb muscles that improves with activity

A

lambert-eaton syndrome

28
Q

what is the diagnostic for lambert-eaton syndrome?

A

EMG

29
Q

what is the treatment for lambert-eaton syndrome? (3)

A

plasmapheresis
+
immunosuppressants
prednisone
azathioprine

30
Q

toxin of Clostridium b. that prevents the release of acetylcholine at the neuromuscular junctions and autonomic synapses

A

botulism

31
Q

a patient with botulism will most likely have what in their history?

A

hx of ingestion of possibly contaminated canned food 72 hrs prior

32
Q

a patient presents with diplopia, dilated pupils, ptosis, facial weakness, dysphagia, respiratory difficulty, limb weakness, and postural hypotension. what are they likely experiencing?

A

botulism

33
Q

what will likely be seen in botulism if we do an electrophysiological study?

A

increased muscle response to fast rate stimulation

34
Q

what is the treatment for botulism?

A

3,4 diaminopyridine (K channel blocker)

Neurology (22 decks)

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