Myasthenia Gravis Flashcards

1
Q

Myasthenia Gravis is an a________ response against…

A

autoimmune
NMJ post-synaptic receptors

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2
Q

What type of hypersensitivity reaction is myasthenia gravis?

A

Type 2

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3
Q

True or false: often patients with Myasthenia Gravis wake up feeling fine and by the end of the day feel weak

A

True

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4
Q

Myasthenia gravis affects ______ muscle

A

skeletal

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5
Q

In myasthenia gravis, what symptoms occur when the extraocular muscles are affected?

A

Diplopia (double vision)
Ptosis

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6
Q

What groups of people are at higher risk of Myasthenia Gravis?

A

20-30 y/o women
and
60-70 y/o men

(unclear why this bimodal distribution)

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7
Q

What does acetylcholine, released from motor neuron, bind to on the muscle cell membrane at a NMJ?

A

Nicotinic acetylcholine receptor

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8
Q

In myasthenia gravis, what cells make the autoantibodies that bind to the nicotinic ACh receptors on muscle cell membrane?

A

B cells

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9
Q

Give a summary of the pathophysiology of Myasthenia gravis

A

Type II hypersensitivity reaction results in B cells producing autoantibodies that bind to the nicotinic ACh receptors on the muscle cell membrane of NMJs, blocking ACh from binding. Therefore, muscle won’t contract.

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10
Q

A minority of people with Myasthenia Gravis produce the harmful antibody called muscle specific receptor t_____ k____ antibodies

A

tyrosine kinase

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11
Q

What do muscle specific receptor tyrosine kinase antibodies attack?

A

Proteins inside muscle cells also leading to destruction of healthy cells

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12
Q

Rarely, a p______ can generate an immune response which results in generation of autoantibodies (Lambert Eaton Syndrome)

A

paraneoplasm

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13
Q

What are symptoms of Myasthenia Gravis?

A

Muscle weakness
Weak eye muscles, diplopia
Ptosis
Myasthenic snarl (difficulty smiling)
Jaw fatigability
Swallowing difficulty
Speech fatigability

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14
Q

What worsens the muscle weakness in Myasthenia Gravis?

A

Exertion (better with rest)

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15
Q

Where do symptoms in Myasthenia Gravis start?

A

In head and neck and goes down to lower body

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16
Q

What condition presents very similarly to Myasthenia Gravis?

A

Lambert Eaton Syndrome

17
Q

What happens in Lambert Eaton Syndrome?

A

There is autoimmunity against pre-synaptic calcium channels

18
Q

True or false: Lambert Eaton Syndrome improves with exertion

A

True

19
Q

Lambert Eaton Syndrome is associated with…

A

small cell lung carcinoma

20
Q

How is Lambert Eaton Syndrome treated?

A

Steroids like prednisolone
and immunosuppression like IV Ig

21
Q

Where do symptoms in Lambert Eaton Syndrome start?

A

Starts in extremities, then head and neck with autonomic involvement (eg. dry eyes and mouth), goes down to lower body

22
Q

What could happen in a myasthenic crisis?

A

Acute symptoms worsening with a decreased function of breathing muscles

23
Q

How do you treat respiratory weakness in a Myasthenic crisis?

A

Plasma exchange and immunosuppression (IV Ig)

24
Q

How is Myasthenia Gravis managed?

A

1st line = Acetylcholinesterase inhibitors:
Neostigmine or Pyridostigmine

Immunosuppressive drugs:
Prednisone

Surgical removal of thymus (unknown exactly why, may be to do with T-helper cells originating in thymus help the B cells to make autoantibodies)

Last resort = Rituximab (monoclonal antibody against B cells)

25
Q

How do acetylcholinesterase inhibitors work in Myasthenia Gravis?

A

Stop breakdown of ACh so increased concentration of ACh around muscle cells. Helps to counteract effects of ACh receptor antibodies.

26
Q

Why is Prednisolone used in Myasthenia Gravis?

A

Reduces the production of autoantibodies

27
Q

How do you diagnose Myasthenia Gravis?

A

Serology: Anti ACh-R

Edrophonium test: administer edrophonium (rapid acting ACh-ase inhibitor and will be increased muscle power in a few seconds for a few seconds.

28
Q

10-20% patients with myasthenia gravis have a t____

A

thymoma

29
Q

What 3 antibodies can cause myasthenia gravis?

A

ACh-receptor antibodies
Muscle-specific kinase antibodies (MuSK)
low density lipoprotein receptor-related protein 4 (LRP4)