Myasthenia gravis Flashcards

1
Q

What is myasthenia gravis?

A

Myasthenia gravis is an autoimmune disorder resulting in insufficient functioning acetylcholine receptors.

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2
Q

What percentage of myasthenia gravis cases have antibodies to acetylcholine receptors?

A

Antibodies to acetylcholine receptors are seen in 85-90% of cases.

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3
Q

What is the gender prevalence of myasthenia gravis?

A

Myasthenia gravis is more common in women with a ratio of 2:1.

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4
Q

What is the key feature of myasthenia gravis?

A

The key feature is muscle fatigability, where muscles become progressively weaker during periods of activity and slowly improve after periods of rest.

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5
Q

What are some symptoms of myasthenia gravis?

A

Symptoms include extraocular muscle weakness (diplopia), proximal muscle weakness (face, neck, limb girdle), ptosis, and dysphagia.

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6
Q

What percentage of myasthenia gravis patients have thymomas?

A

Thymomas are found in 15% of myasthenia gravis patients.

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7
Q

What autoimmune disorders are associated with myasthenia gravis?

A

Associated autoimmune disorders include pernicious anemia, autoimmune thyroid disorders, rheumatoid arthritis, and systemic lupus erythematosus (SLE).

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8
Q

What percentage of myasthenia gravis patients have thymic hyperplasia?

A

Thymic hyperplasia is seen in 50-70% of myasthenia gravis patients.

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9
Q

What is the most sensitive investigation for myasthenia gravis?

A

Single fibre electromyography has a high sensitivity of 92-100%.

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10
Q

What imaging is used to exclude thymoma in myasthenia gravis?

A

CT thorax is used to exclude thymoma.

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11
Q

What is the typical CK level in myasthenia gravis?

A

CK levels are typically normal in myasthenia gravis.

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12
Q

What antibodies are tested in myasthenia gravis?

A

Antibodies to acetylcholine receptors are positive in around 85-90% of patients; about 40% of remaining patients are positive for anti-muscle-specific tyrosine kinase antibodies.

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13
Q

What is the Tensilon test?

A

The Tensilon test involves IV edrophonium, which temporarily reduces muscle weakness, but is not commonly used anymore due to the risk of cardiac arrhythmia.

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14
Q

What is the first-line treatment for myasthenia gravis?

A

Pyridostigmine is the first-line treatment, which is a long-acting acetylcholinesterase inhibitor.

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15
Q

What immunosuppressive treatments may be used in myasthenia gravis?

A

Immunosuppression may include prednisolone initially, and azathioprine, cyclosporine, or mycophenolate mofetil may also be used.

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16
Q

What surgical procedure may be performed for myasthenia gravis?

A

Thymectomy may be performed as part of the management.

17
Q

What is the management for myasthenic crisis?

A

Management includes plasmapheresis and intravenous immunoglobulins.

18
Q

Are antibodies less commonly seen in ocular myasthenia gravis?

A

Yes, antibodies are less commonly seen in disease limited to the ocular muscles.

19
Q

What is the most common exacerbating factor of myasthenia gravis?

A

Exertion resulting in fatigability, which is the hallmark feature of myasthenia gravis. Symptoms become more marked during the day.

20
Q

Which drugs may exacerbate myasthenia gravis?

A

The following drugs may exacerbate myasthenia: penicillamine, quinidine, procainamide, beta-blockers, lithium, phenytoin, and certain antibiotics (gentamicin, macrolides, quinolones, tetracyclines).