Myasthenia Gravis Flashcards

1
Q

Define MG

A

An autoimmune disorder of the neuromuscular

Fluctuating motor weakness

Ocular, bulbar, limb, respiratory muscles

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2
Q

What does myasthenia gravis affect

A

Bulbar nerves of the medulla

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3
Q

What are the consequences of bulbar damage

A

Difficulty:
Speaking
Swallowing
Dysphasia

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4
Q

What causes MG

A

Antibody mediators

Immunological attack at proteins in the post-synaptic membrane

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5
Q

What is ocular MG

A

Muscle weakness
Limited to the eyelids and extraocular muscles

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6
Q

What is generalized MG

A

Involve variable combinations of weakness:

Bulbar
Limbs
Respiratory
Facial
Weakness of neck

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7
Q

What are the pulmonary consequences of MG

A

Respiratory failure
Atelectasis
Alveolar consolidation poor control of secretions

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8
Q

What are the pulmonary consequences of MG

A

Respiratory failure
Atelectasis
Alveolar consolidation poor control of secretions

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9
Q

What is the typical course of MG

A

Onset can be:
Subtle
Intermittent
Sudden

Head to toe

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10
Q

When do women show signs of MG

A

2-3rd decade

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11
Q

When do women show signs of MG

A

2-3rd decade

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12
Q

What antibody disrupts transmission of ACh

A

IgG

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13
Q

What does Ptosis mean for

A

Drooping eye

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14
Q

What does diploid mean for

A

Double vision

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15
Q

What does disarthria mean for

A

People cannot articulate words

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16
Q

What does dysphasia mean

A

Difficulty swallowing

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17
Q

Explain the ice pack test for MG

A

Only done with ocular MG

Transmission is better at lower temperatures

Let it sit for 2 minutes

Sensitivity is 80%

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18
Q

What MG test is outdated

A

Edrophonium test (Tensilon)

19
Q

What did the Edrophonium test do

A

Confirm MG

Distinguished between myasthenic crisis vs cholinergic crisis

Was unreliable

20
Q

What did you use if you had adverse effects to Edrophonium test

21
Q

What is the seropositive test

A

Test the blood to see if they have:

AChR antibodies

MuSK antibodies

Only 50% with ocular

22
Q

Why does MuSK stand for

A

Muscle-specific receptor tyrosine kinase

23
Q

What is the seronegative MG test

A

90% of generalized MG patients lack AChR and MuSK antibodies

24
Q

What are the three electrodiagnostic studies for MG

A

Nerve Conduction Studies

Electromyography

Single-fiber electromyography

25
Explain the single-fiber electromyography (SFEMG)
Most sensitive test available Special needle records action potential of two muscle fibers innervated by the same motor axon When stimulated, second fiber will signal after causing a “jitter” Not widely available
26
Describe the repetitive nerve stimulation (RNS) test for MG
Stimulate the motor nerve 6-10x Normal response: no change in compound muscle action potential amp MG response: progressive decline in CAMP amp after 4-5x (>10% decline = +)
27
Describe electomyography
Evaluates electric activity of muscle at rest and with voluntary action
28
Why is the thymus assessed with MG
Antibodies are produced by thymus A thymoma or thymus mass may cause it CT or MRI
29
What does a CXR look like with MG
Normal, unless atelectasis
30
What is MG crisis
There form of an exacerbation
31
How often should NIF or VC be done with MG
Q4 In severe situations, Q2
32
What do you do when monitoring MG for respiratory failure
VC NIF SpO2 ABG
33
What NIF indicates Respiratory failure with MG
More positive than -30cmH2O
34
What O2 saturation indicates respiratory failure with MG
<92%
35
What MEP indicates respiratory failure with MG
<40cmH2O
36
What FVC indicates respiratory failure with MG
<20cmH2O or a decrease >30% prior
37
What Respiratory Rate indicates respiratory failure with MG
>30 breaths/min
38
What are treatment strategies for MG
Increase ACh to receptors Immunosuppressive and immunomodulatory agent (plasma exchange) Surgery
39
What medication increases the ACh to junction
Pyridostigmine (mestinon) Also inhibits acetylcholinesterase Effective in mild cases
40
What immunosuppressant is first used for MG
Used when symptomatic on acetylcholinesterase Initially glucocorticoid until safer med is available
41
What are ideal immunosuppressants meds for MG
Azathioprine Mycophenolate mofetl Cyclosporine
42
What immunomodulating treatment is used for MG
Plasma exchange (removal of AChR antibodies) Intravenous immune globulin (IVIG)
43
What are the additional chronic managements for MG
Avoids meds that exacerbate MG Seek vaccination