Myasthenia Gravis 

Myasthenia gravis is an autoimmune disorder of the postsynaptic neuromuscular junction - associated with thymic tumors, paraneoplastic effects of SCC or hodgkin lymphoma. Autoimmune: thyroid, Sjogren syndrome, rheumatoid arthritis, SLE

Clinical picture - fluctuating degree/variable degree of weakness in:

1. Ocular- 50%. Ptosis, diplopia

2. Bulbar- 15%. Dysphagia, dysarthria, hypophonia, fatiguable chewing, loss of smile

3. Neck "dropping head syndrome"

3. Limb - proximal weakness, arms > legs

4. Respiratory muscles- "myasthenia crises" Respiratory insuffciency and life threatening

Diagnosis:

 - Clinical Diagnosis

 - Bedside: ice pack test/ Edrophonium test

 - Cogan sign

 - Peek sign

Electrophysiologic confirmation:

 - Repetitive nerve stimulation

 - Single fiber electromyography

Labs:

 - AchR antibodies- first step in immunologic assay

 - MuSK antibodies

 - LRP4 antibodies

Rx:

 - Symptomatic treatment: acetylcholinesterase inhibition

 - Chronic immunosuppressive therapies: steroids and NSAIDS

 - Immunomodulating treatments: IVIG and Therapeutic plasma exchange

 - Surgical Treatment: Thymectomy



- Dr. Ravi Singh @rav7ks



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