Myasthenia Gravis Flashcards
1
Q
Myasthenia Gravis is:
A
- charaterized by severe muscle weakness
- autoimmune disorder
- antibodies are produced agianst the nicotinic receptors
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
3
Q
Myasthenia Gravis in the Oculomotor muscles
A
- double vision (diplopia)
4
Q
Myasthenia Gravis in the Elevator muscles of the eye lid
A
- lid drooping (ptosis)
5
Q
Myasthenia Gravis in Oropharyngeal muscles
A
- difficulty swallowing
6
Q
Myasthenia Gravis in Facial muscles
A
- difficulty speaking, and smiling
7
Q
Myasthenia Gravis in Trunk and Limb muscles
A
- diaphragm becomes weak (major concern)
8
Q
Seropositive Myasthenia Gravis
A
- antibodies to nicotinic receptors in the serum
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
10
Q
Diagnostic tests for Myasthenia Gravis
A
- Musclar Power test
- Edrophonium (Tensilon) test
- Give tubocurarine at 1/20 the usual therapeutic dose
- Detection of circulating antibodies to AChR, and MuSK
- Measure ACh receptor density from a muscle biopsy (uses radiolabelled alpha-bungarotoxin)
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
12
Q
Tubocurarine at 1/20 therapeutic dose test
A
- positive if it decrease muscle strength
Only identifies a defect in neuromuscluar transmission
13
Q
Antibody test
A
- finds the cause of muscle weakness
- best diagnostic test
14
Q
Drug Treatment options
A
- Oral Acetylcholinesterase inhibitors
- Corticosteroid therapy
- Immunosuppresive drugs
- Plasms Exchange
- Intravenous Immunoglobulin Infusion
- Thymectomy
15
Q
oral acetylcholinesterase inhibitor
A
Pyridostigmine (Mestinon)