Myasthenia Gravis Flashcards

1
Q

Myasthenia gravis is a condition affecting the neuromuscular junction of skeletal muscles. What is the cause of this disease?

1 - idiopathic
2 - autoimmune
3 - metabolic
4 - vascular

A

2 - autoimmune
- autoantibodies target nicotinic acetylcholine receptors on post synapse
- B and T cells are involved

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2
Q

What is the prevalence of myasthenia gravis?

1 - 1.5-36.7 cases per 100,000
2 - 105-360 cases per 100,000
3 - 1500-3600 cases per 100,000
4 - 15,00-36,000 cases per 100,000

A

1 - 1.5-36.7 cases per 100,000
- this is a rare condition

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3
Q

Are men or women more at risk of myasthenia gravis?

A
  • women
  • typically aged 20-30 y/o
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4
Q

What type of hypersensitivity is myasthenia gravis classified as?

1 - type 1 hypersensitivity
2 - type 2 hypersensitivity
3 - type 3 hypersensitivity
4 - type 4 hypersensitivity

A

2 - type 2 hypersensitivity
- also called cytotoxic hypersensitivity
- T cells and complement pathway lead to cell damage, inflammation and apoptosis

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5
Q

In myasthenia gravis is it just the acetylcholine receptors on the post synapse that are affected?

A
  • no
  • some patients produce another antibody that can enter skeletal muscle leading to apoptosis of skeletal muscle cells
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6
Q

In myasthenia gravis the acetylcholine receptors on the post synapse or skeletal muscle cells can be damaged, inducing apoptosis due to autoantibodies. What has also been identified as a cause of myasthenia gravis?

1 - alcohol
2 - smoking
3 - metabolic syndrome
4 - paraneoplastic syndrome

A

4 - paraneoplastic syndrome
- bronchiogenic carcinoma or thymic neoplasm are most common causes
- malignancy induces and immune response and auto-antibodies, which subsequently targets the NMJ

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7
Q

Myasthenia gravis can present with all of the following, but which is the tell tale sign?

1 - ptosis
2 - muscular weakness with fatigue
3 - bulbar (swallowing, chewing)
4 - facial/neck weakness
5 - limb girdle and trunk

A

2 - muscular weakness with fatigue
- exhausted in the evenings

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8
Q

Which of the following are clinical signs of myasthenia gravis?

1 - dysphonia upon counting (slurred)
2 - ptosis and diplopia (double vision)
3 - myasthenia snarl when smiling
4 - peak signs of orbicularis (eyes opening despite you closing them)
5 - general weakness
6 - all of the above

A

6 - all of the above

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9
Q

Although any skeletal muscles can be affected, which is often the most common?

1 - muscles of hands
2 - extra-ocular muscles
3 - respiratory muscles
4 - cardiac muscles

A

2 - extra-ocular muscles
- common to see ptosis (dropping) and diplopia (double vision)

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10
Q

There are some common exacerbating factors of myasthenia gravis. Which of the following is NOT one of these?

1 - pregnancy
2 - low Na+
3 - exercise
4 - gentamicin
5 - beta blockers
6 - opiates

A

2 - low Na+
- low K+ can make patients worse
- the rest of these medications can act as neuromuscular blockade and make myasthenia gravis symptoms worse

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11
Q

All of the following are typical differentials for myasthenia gravis, EXCEPt which one?

1 - polymyositis
2 - myopathies
3 - connective tissue disorders (SLE)
4 - stroke
5 - botulism
6 - Lambert-Eaton myasthenic syndrome

A

4 - stroke

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12
Q

Patients who are suspected of having myasthenia gravis should be screened for ermatomyositis, polymyositis, systemic lupus erythematosus, Addison’s disease, Guillain-Barré syndrome and juvenile rheumatoid arthritis. What do all of these have in common?

1 - higher incidence in women
2 - occur at a younger age
3 - all autoimmune disease
4 - all of the above

A

3 - all autoimmune disease

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13
Q

Which of the following is NOT a typically used test when trying to diagnose a patient with myasthenia gravis?

1 - ACh antibodies
2 - CK-MB
3 - EMG (decremental muscle response)
4 - CT to exclude malignancy (thyoma or bronchiogenic carcinoma)
5 - thyroid function

A

2 - CK-MB

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14
Q

Can myasthenia gravis be dangerous and a risk to life?

A
  • yes
  • myasthenia crisis
  • respiratory and/or cardiac depression
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15
Q

What is the typical 1st line medication for patients with myasthenia gravis?

1 - Acetylcholinesterase inhibitors
2 - NMDA receptor antagonist
3 - Dopamine agonist
4 - Anticholinergics

A

1 - Acetylcholinesterase inhibitors
- increase ACh in synaptic cleft
- core drug for is Donepezil
- drug referenced in lecture was Pyridostigmine

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16
Q

Acetylcholinesterase inhibitors are given as a symptomatic treatment for patients with myasthenia gravis. Which of the following is NOT typically a side effect of acetylcholinesterase inhibitors?

1 - diarrhoea
2 - abdominal pain common
3 - parasympathetic muscarinic
4 - tachycardia

A

4 - tachycardia
- typically causes bradycardia

17
Q

Acetylcholinesterase inhibitors is the typical 1st line medication for patients with myasthenia gravis. However, if a patient has a relapse or present with a myasthenia gravis crisis, what medication should they be given?

1 - Anticholinergics
2 - NMDA receptor antagonist
3 - Dopamine agonist
4 - Steroid

A

4 - Steroid
- specifically prednisolone
- need to use with caution as this can exacerbate myasthenia gravis symptoms

18
Q

Patients with myasthenia gravis can be given immunoglobulins via IV as part of immuno modulation. What is immunoglobulins as a treatment?

1 - patients are vaccinated to improve immune system
2 - patients have monoclonal antibodies made for them
3 - patients are given a mixture of antibodies (typically IgG and IgM)
4 - all of the above

A

3 - patients are given a mixture of antibodies (typically IgG and IgM)
- similar to a blood transfusion

  • Anaphylaxis (IgA deficiency, small risk)
  • Mostly immunomodulatory
  • Highly viscous
  • Prothrombotic especially VTE
19
Q

Patients with myasthenia gravis can be given plasmapheresis as part of immuno modulation. What is plasmapheresis as a treatment?

1 - patients are vaccinated to improve immune system
2 - patients have monoclonal antibodies made for them
3 - patients are given a mixture of antibodies (typically IgG and IgM)
4 - patients own antibodies are taken out and replaced with blood products

A

4 - patients own antibodies are taken out and replaced with blood products

  • removes plasma and blood products, specifically the antibodies that are attacking ACh receptors
  • need to monitor coagulation factors as these can be lost in this process
20
Q

If a patient has a thymoma, can a thyroidectomy have any beneficial effect?

A
  • yes
  • has shown benefits of reducing muscke weakness even in patients without a thymoma
  • potentially linked with reduced T cell production so less cytotoxic T cells