Myasthenia Gravis

Question 1

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

Answer 1

anti-acetylcholinesterase receptor (AChRs)

Question 2

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

Answer 2

myasthenia gravis

Question 3

Myasthenia gravis weakness and fatigue improves with rest

a. true
b. false

Answer 3

a. true

Question 4

Ocular symptoms of myasthenia gravis occur __ in the course

a. early
b. late

Answer 4

a. early

Question 5

Facial signs of myasthenia gravis?

Answer 5

ptosis (drooping eyelid)

facial weakness

Question 6

4 tests for myasthenia gravis?

Answer 6

ice pack test
autoantibodies test
electrodiagostic test
anti cholinesterase test

Question 7

What is edrophonium?

Answer 7

anti cholinesterase test for myasthenia gravis

Question 8

What are Anti-AChR and Anti-Must?

Answer 8

autoantibody tests for myasthenia gravis

Question 9

There is __ reduction in AChR in the cold

a. more
b. less

Answer 9

b. less

less reduction in AChR and reduced activity of AChE

ice pack test

Question 10

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

Answer 10

thymus

Question 11

Thymectomy is an emergency procedure

a. true
b. false

Answer 11

b. false

ELECTIVE, not emergency

Question 12

Thymectomy is not used for pts with __ or __ antibodies

Answer 12

MuSK, LRP4

Question 13

What anti cholinesterase inhibitor is used for MG?

Answer 13

pyridostigmine

Question 14

Pyridostigmine starts working how fast?

Answer 14

15-30mins

lasts 3-4hrs

Question 15

Which drug is used for MG ion pts with diplopia?

Answer 15

pyridostigmine

Question 16

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

Answer 16

prednisone

Question 17

Use prednisone for MG for _how long before tapering?

Answer 17

one month

Question 18

4 immunosuppressants used for MG?

Answer 18

azathioprine
cyclosporine
tacrolimus
mycophenolate

Question 19

Which MG drug blocks purine synthesis pathway/

Answer 19

azathioprine

Question 20

There is increased risk of infection with azathioprine

a. true
b. false

Answer 20

a. true

Question 21

Clinical response to azathioprine for MG may take how long?

Answer 21

up to a year

Question 22

What is BBW for azathioprine?

Answer 22

chronic suppression increases risk of malignancy

Question 23

Pts with what deficiency have high risk for severe myelosuppression?

Answer 23

TPMT

Question 24

Avoid what drug with azathioprine?

Answer 24

allopurinol

increased risk of bone marrow suppression

Question 25

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

Answer 25

cyclosporine

Question 26

Clinical response to cyclosporine may take how long?

Answer 26

1-3 months with maximum effect apparent at 7 months

Question 27

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

Answer 27

cyclosporine

Question 28

Grapefruit juice __ the concentration of cyclosporine a. increases b. decreases

Answer 28

a. increases

Question 29

Tacrolimus blocks what?

Answer 29

T-cell activation

Question 30

Tacrolimus can cause __ a. hypokalemia b. hyperkalemia

Answer 30

b. hyperkalemia

Question 31

Clinical response with tacrolimus for MG may take how long?

Answer 31

6-12 months

Question 32

Therapeutic range for tacrolimus is __ ng/L

Answer 32

5-15

Question 33

Which MG med can cause QTc prolongation?

Answer 33

tacrolimus

Question 34

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

Answer 34

tacrolimus

Question 35

What is mycophenolate MOA?

Answer 35

blocks purine synthesis blocks proliferation of B and T cells

Question 36

Clinical response to mycophenolate may take up to __ months

Answer 36

12 max effet 1-2 years

Question 37

Mycophenolate is widely used for MG for __ term Tx a. long b. short

Answer 37

a. long

Question 38

Mycophenolate has BBW for pregnancy loss during which trimester?

Answer 38

first

Question 39

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

Answer 39

mycophenolate also for increased risk of infection

Question 40

Eculizumab for MG binds to what complement protein?

Answer 40

protein 5 blocks membrane attack complex

Question 41

Which MG med is used for generalized refractory pts/

Answer 41

eculizumab

Question 42

Monitor for signs of meningococcal infection with which MG med?

Answer 42

eculizumab vaccination required 2 weeks prior to initiation

Question 43

Which med is used for severe refractory MG?

Answer 43

rituximab

Question 44

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

Answer 44

rituximab

Question 45

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

Answer 45

rituximab also for fatal infusion rxn

Question 46

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

Answer 46

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

Question 47

What is a Sx of myasthenia crisis?

Answer 47

difficulty breathing

Question 48

2 Tx for myasthenia crisis?

Answer 48

plasmapheresis | IVIG

Question 49

Plasmapheresis dose?

Answer 49

5 exchanges over 10-14 days

Question 50

Do not use plasmapheresis in pts with __ shock

Answer 50

septic

Question 51

__ is CI in pts with hypercoagulable state or renal failure a. plasmapheresis b. IVIG

Answer 51

b. IVIG

Question 52

___ decreases acetylcholine from from being released

Answer 52

magnesium

Question 53

What med can cause MG?

Answer 53

penicillamine

Question 54

What class of BP meds has drug interactions in MG?

Answer 54

beta blockers

Question 55

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

Answer 55

Guillain-Barre Syndrome

Question 56

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)

Answer 56

a. acute inflammatory demyelinating polyneuropathy (AIDP)

Question 57

What does GBS damage?

Answer 57

myelin sheath on axone of peripheral nerves

Question 58

2 vaccinations that can cause GBS?

Answer 58

influenza | rabies

Question 59

What bacteria can cause GBS?

Answer 59

Campylobacter

Question 60

GBS pts have __ CSF and __ WBCs a. increased, increased b. decreased, increased c. increased, decreased

Answer 60

c. increased, decreased

Question 61

Where do GBS Sx usually start?

Answer 61

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

Question 62

GBS pts have absent or diminished __ reflexes in weak limbs

Answer 62

deep tendon

Question 63

There is no known cure for GBS a. true b. false

Answer 63

a. true

Question 64

GBS Tx should be initiated within __ weeks

Answer 64

two

Question 65

What is Tx for GBS?

Answer 65

plasma exchange or high dose IVIG

Question 66

GBS plasma exchange should begin within __ days of Sx onset

Answer 66

7

Question 67

Which vaccine has no increased risk for GBS? a. influenza b. meningococcal c. rabies

Answer 67

b. meningococcal

Question 68

Which two vaccines are contraindicate within 6 weeks of GBS?

Answer 68

influenza | Tdap or Td

Question 69

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

Answer 69

6

Question 70

There is no cure for myasthenia gravis a. true b. false

Answer 70

a. true

Question 71

There is no cure for Guillain-Barre syndrome a. true b. false

Answer 71

a. true

Question 72

Therapeutic range of cyclosporine is __ ng/L

Answer 72

150-200

Question 73

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

Answer 73

2