Myasthenia Gravis Flashcards

1
Q

Myasthenia Gravis is:

A
  • charaterized by severe muscle weakness
  • autoimmune disorder
  • antibodies are produced agianst the nicotinic receptors
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2
Q

Symptoms of Myasthenia Gravis:

A

muscle weakness is:

  • aggravated by exercise, but improved by rest
  • usually less evident on awakening and get worse as the day progresses
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3
Q

Myasthenia Gravis in the Oculomotor muscles

A
  • double vision (diplopia)
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4
Q

Myasthenia Gravis in the Elevator muscles of the eye lid

A
  • lid drooping (ptosis)
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5
Q

Myasthenia Gravis in Oropharyngeal muscles

A
  • difficulty swallowing
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6
Q

Myasthenia Gravis in Facial muscles

A
  • difficulty speaking, and smiling
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7
Q

Myasthenia Gravis in Trunk and Limb muscles

A
  • diaphragm becomes weak (major concern)
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8
Q

Seropositive Myasthenia Gravis

A
  • antibodies to nicotinic receptors in the serum
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9
Q

Seronegative Myasthenia Gravis

A
  • antibodies against muscle-specific kinase (MuSK) (membrane signalling element)
    OR
  • a mutation in Rapsyn (a membrane signalling protein) - NOT autoimmune
    OR
  • congenital due to an inherited deficiency in ACh receptors - NOT autoimmune
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10
Q

Diagnostic tests for Myasthenia Gravis

A
  1. Musclar Power test
  2. Edrophonium (Tensilon) test
  3. Give tubocurarine at 1/20 the usual therapeutic dose
  4. Detection of circulating antibodies to AChR, and MuSK
  5. Measure ACh receptor density from a muscle biopsy (uses radiolabelled alpha-bungarotoxin)
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11
Q

Edrophonium (Tensilon) Test

A
  • it is a short acting acetylcholinesterase inhibitor
  • given iv
  • positive if there is a marked increase in muscle strength within 1 minute
    Only identifies a defect in neuromusclar transmission
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12
Q

Tubocurarine at 1/20 therapeutic dose test

A
  • positive if it decrease muscle strength

Only identifies a defect in neuromuscluar transmission

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13
Q

Antibody test

A
  • finds the cause of muscle weakness

- best diagnostic test

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14
Q

Drug Treatment options

A
  • Oral Acetylcholinesterase inhibitors
  • Corticosteroid therapy
  • Immunosuppresive drugs
  • Plasms Exchange
  • Intravenous Immunoglobulin Infusion
  • Thymectomy
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15
Q

oral acetylcholinesterase inhibitor

A

Pyridostigmine (Mestinon)

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16
Q

Prednisone

A

Corticosteroid

17
Q

Corticosteroid Therapy

A
  • used for patients with myasthenia gravis confined to eye muscles or generalized muscle weakness
  • used for patients who do not respond to conventional treatments
  • titrated dose
  • when remission is acheived dose is slowly lowered to the effective minimal dose
18
Q

Adverse effect of Corticosteroid therapy

A
  • weight gain, cataracts, diabetees, insomnia, mood change (depression), and osteoporosis
19
Q

Depression with Myasthenia Gravis

A
  • depression can be mistaken for Myasthenia Gravis
  • during corticosteroid therapy dose will be increased, but increasing the dose will worsen the depression
  • then around we go
20
Q

Immunosuppressive drugs

A
Azathioprine
Cyclosporine
Cyclophosphamide
- global decrease in immune repsonse
- for the most serious or resistant cases
21
Q

Plasma Exchange (Plasmapheresis)

A
  • removes circulating antibodies
  • separation of blood cells from plasma cells
  • only temporary improvement
  • used for myasthemic crisis
22
Q

Myasthenic Crisis

A
  • Severe Muscle Weakness event
  • severe and sudden exacerbation of the existing disease
  • affect on breathing is the main concern
23
Q

Intravenouse Immunoglobulin Infusion

A
  • treatment for myasthemic crisis
24
Q

Thymectomy

A
  • removal of the thymus gland in cases of tumor
  • option for moderate to severe seropositive myasthenia gravis who are inadequately controlled by drug therapy
  • for patients under 45
  • miximum improvement occurs in the first year after surgery
25
Q

Cholinergic Crisis

A
  • results from too musch ACh

- symptoms are like anticholinesterase poisoning

26
Q

Symptoms of Cholinergic Crisis

A

DUMMBELSSS

Diarrhea, Urintaion, Miosis, Muscle Weakness, Bronchospasm, Excitation, Lacrimation, Seizures, Sweating, Salivation

27
Q

How to Differentiate between Chlonergic and Myasthenic Crisis

A
  • give the patient edrophonium
  • if it is a cholinergic crisis it will make it worse
  • if it is a myasthenic crisis it will make it better