Myasthenia Gravis Flashcards

1
Q

Myasthenia gravis is an autoimmune disorder caused by what type of antibodies?

A

anti-acetylcholinesterase receptor (AChRs)

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2
Q

__ is a neuromuscular junction disorder characterized by weakness and fatigue of the skeletal muscles

A

myasthenia gravis

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3
Q

Myasthenia gravis weakness and fatigue improves with rest

a. true
b. false

A

a. true

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4
Q

Ocular symptoms of myasthenia gravis occur __ in the course

a. early
b. late

A

a. early

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5
Q

Facial signs of myasthenia gravis?

A

ptosis (drooping eyelid)

facial weakness

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6
Q

4 tests for myasthenia gravis?

A

ice pack test
autoantibodies test
electrodiagostic test
anti cholinesterase test

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7
Q

What is edrophonium?

A

anti cholinesterase test for myasthenia gravis

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8
Q

What are Anti-AChR and Anti-Must?

A

autoantibody tests for myasthenia gravis

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9
Q

There is __ reduction in AChR in the cold

a. more
b. less

A

b. less

less reduction in AChR and reduced activity of AChE

ice pack test

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10
Q

What gland can be removed for non-pharm therapy of myasthenia gravis?

A

thymus

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11
Q

Thymectomy is an emergency procedure

a. true
b. false

A

b. false

ELECTIVE, not emergency

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12
Q

Thymectomy is not used for pts with __ or __ antibodies

A

MuSK, LRP4

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13
Q

What anti cholinesterase inhibitor is used for MG?

A

pyridostigmine

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14
Q

Pyridostigmine starts working how fast?

A

15-30mins

lasts 3-4hrs

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15
Q

Which drug is used for MG ion pts with diplopia?

A

pyridostigmine

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16
Q

___ is used in pts who have not met Tx goals after using pyridostigmine

A

prednisone

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17
Q

Use prednisone for MG for _how long before tapering?

A

one month

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18
Q

4 immunosuppressants used for MG?

A

azathioprine
cyclosporine
tacrolimus
mycophenolate

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19
Q

Which MG drug blocks purine synthesis pathway/

A

azathioprine

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20
Q

There is increased risk of infection with azathioprine

a. true
b. false

A

a. true

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21
Q

Clinical response to azathioprine for MG may take how long?

A

up to a year

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22
Q

What is BBW for azathioprine?

A

chronic suppression increases risk of malignancy

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23
Q

Pts with what deficiency have high risk for severe myelosuppression?

A

TPMT

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24
Q

Avoid what drug with azathioprine?

A

allopurinol

increased risk of bone marrow suppression

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25
Q

__ blocks production and release of interleukin II and inhibits IL-2 induced activation of T cells

A

cyclosporine

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26
Q

Clinical response to cyclosporine may take how long?

A

1-3 months

with maximum effect apparent at 7 months

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27
Q

Which MG drug has BBW for increased risk of infection, risk of HTN with increased doses/duration, increased risk of malignancy nephrotoxicity?

A

cyclosporine

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28
Q

Grapefruit juice __ the concentration of cyclosporine

a. increases
b. decreases

A

a. increases

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29
Q

Tacrolimus blocks what?

A

T-cell activation

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30
Q

Tacrolimus can cause __

a. hypokalemia
b. hyperkalemia

A

b. hyperkalemia

31
Q

Clinical response with tacrolimus for MG may take how long?

A

6-12 months

32
Q

Therapeutic range for tacrolimus is __ ng/L

A

5-15

33
Q

Which MG med can cause QTc prolongation?

A

tacrolimus

34
Q

Which MG med has BBW for risk of infection and risk of malignancy?

A

tacrolimus

35
Q

What is mycophenolate MOA?

A

blocks purine synthesis

blocks proliferation of B and T cells

36
Q

Clinical response to mycophenolate may take up to __ months

A

12

max effet 1-2 years

37
Q

Mycophenolate is widely used for MG for __ term Tx

a. long
b. short

A

a. long

38
Q

Mycophenolate has BBW for pregnancy loss during which trimester?

A

first

39
Q

Which MG med has BBW for risk of lymphoma or skin malignancy?

A

mycophenolate

also for increased risk of infection

40
Q

Eculizumab for MG binds to what complement protein?

A

protein 5

blocks membrane attack complex

41
Q

Which MG med is used for generalized refractory pts/

A

eculizumab

42
Q

Monitor for signs of meningococcal infection with which MG med?

A

eculizumab

vaccination required 2 weeks prior to initiation

43
Q

Which med is used for severe refractory MG?

A

rituximab

44
Q

Which med is considered as an early treatment option in patients with MuSK antibody positive MG who have an unsatisfactory response to initial immunotherapy?

A

rituximab

45
Q

Which MG med has a BBW for reactivation of hep B?

A

rituximab

also for fatal infusion rxn

46
Q

__ is defined as an exacerbation of weakness sufficient to endanger life

A

myasthenia crisis

Requires admission to an intensive care or step-down unit to monitor for or manage respiratory failure and bulbar dysfunction

47
Q

What is a Sx of myasthenia crisis?

A

difficulty breathing

48
Q

2 Tx for myasthenia crisis?

A

plasmapheresis

IVIG

49
Q

Plasmapheresis dose?

A

5 exchanges over 10-14 days

50
Q

Do not use plasmapheresis in pts with __ shock

A

septic

51
Q

__ is CI in pts with hypercoagulable state or renal failure

a. plasmapheresis
b. IVIG

A

b. IVIG

52
Q

___ decreases acetylcholine from from being released

A

magnesium

53
Q

What med can cause MG?

A

penicillamine

54
Q

What class of BP meds has drug interactions in MG?

A

beta blockers

55
Q

__ is a rare neurologic disorder where the immune system attacks the peripheral nervous system, particularly the brain and spinal chord

A

Guillain-Barre Syndrome

56
Q

Which is the most common type of GBS?

a. acute inflammatory demyelinating polyneuropathy (AIDP)
b. acute motor axonal neuropathy (AMAN)
c. acute motor sensory axonal neuropathy (AMSAN)
d. miller Fisher syndrome (MFS)

A

a. acute inflammatory demyelinating polyneuropathy (AIDP)

57
Q

What does GBS damage?

A

myelin sheath on axone of peripheral nerves

58
Q

2 vaccinations that can cause GBS?

A

influenza

rabies

59
Q

What bacteria can cause GBS?

A

Campylobacter

60
Q

GBS pts have __ CSF and __ WBCs

a. increased, increased
b. decreased, increased
c. increased, decreased

A

c. increased, decreased

61
Q

Where do GBS Sx usually start?

A

legs

recent onset within days to at most 4 weeks of symmetrical weakness, usually starting in legs

62
Q

GBS pts have absent or diminished __ reflexes in weak limbs

A

deep tendon

63
Q

There is no known cure for GBS

a. true
b. false

A

a. true

64
Q

GBS Tx should be initiated within __ weeks

A

two

65
Q

What is Tx for GBS?

A

plasma exchange or high dose IVIG

66
Q

GBS plasma exchange should begin within __ days of Sx onset

A

7

67
Q

Which vaccine has no increased risk for GBS?

a. influenza
b. meningococcal
c. rabies

A

b. meningococcal

68
Q

Which two vaccines are contraindicate within 6 weeks of GBS?

A

influenza

Tdap or Td

69
Q

Influenza and Tdap/Td are CI within __ weeks of GBS

A

6

70
Q

There is no cure for myasthenia gravis

a. true
b. false

A

a. true

71
Q

There is no cure for Guillain-Barre syndrome

a. true
b. false

A

a. true

72
Q

Therapeutic range of cyclosporine is __ ng/L

A

150-200

73
Q

IVIG for MG is given __g/kg administered over 2-5 days

A

2