Integrated 19 subject Revision (#INR-61):

Myasthenia Gravis
Myasthenia Gravis: Myasthenia Gravis:
Normal neuromuscular junction
• Most common Neuro Muscular Junction disorder. Impaired neuromuscular junction
• Female 30–40 years.
• Autoimmune disorder with autoantibodies to
postsynaptic Ach (Acetylcholine) receptor.
• Fluctuating muscle weakness and fatigability that
worsens towards the end of day.
• Associated with Thymic hyperplasia > Thymoma
(Harrison 21ed,pg 3510)

Normal Muscle Muscle weakness

Dr. Devesh Mishra

MBBS, MD, AIIMS
Myasthenia Gravis: Myasthenia Gravis:
Normal neuromuscular junction
• Most common Neuro Muscular Junction disorder. Impaired neuromuscular junction
• Female 30–40 years.
• Autoimmune disorder with autoantibodies to
postsynaptic Ach (Acetylcholine) receptor.
• Fluctuating muscle weakness and fatigability that
worsens towards the end of day.
• Associated with Thymic hyperplasia > Thymoma
(Harrison 21ed,pg 3510)

Normal Muscle Muscle weakness

Ocular MG:
Diplopia (Double vision)
• Ptosis (Droopy upper eyelid)
• Diplopia (double vision).
Generalized MG:
• Bulbar (dysarthria, dysphagia, fatigable chewing)
• Facial (expressionless).
• Respiratory muscles (weakness).

Ptosis (Droopy upper eyelid)

Diagnostic tests: Repetitive nerve stimulation test
1) Repetitive nerve stimulation:
• Compound muscle action potential (CMAP) A) Normal subjects:
amplitude decrement of >10% at 3 Hz: highly
probable.
2) Single-fiber electromyography (SFEMG): B) Myasthenia gravis (MG):
• Postsynaptic neuromuscular junction disorder.
• Most sensitive diagnostic test for MG and it
shows blocking and jitter, with normal fiber
density.
• Confirmatory, but not specific. C) Lambert–Eaton syndrome (LES):
• Presynaptic neuromuscular junction disorder.

Single-fiber electromyography (SFEMG):

 3) Tensilon (Edrophonium) test:
Tensilon (Edrophonium) test
• Tensilon (acetylcholinesterase inhibitor) that
prolongs the presence of ACh at neuromuscular
junction.
• Positive test results in an immediate increase in
the strength of affected muscles.
4) Ice pack test:
• Used in ptosis.
• Ice pack over eyelids for 2 minutes improves
ptosis.

Ice pack test:

Left drooping eyelid Ice pack on left drooping eyelid Improvement of Ptosis
5) Immunologic assay:
• MuSK ( Muscle Specific Kinase) antibodies.
• LRP4 (LDL Receptor related Protein 4) antibodies.

6) For ocular or cranial MG:

• Exclude intracranial lesions by CT or MRI.

Treatment:
• Pyridostigmine (acetylcholinesterase inhibitor).
• Surgery is thymectomy. Immunologic assay :
• MuSK ( Muscle Specific Kinase) antibodies.
Mnemonic : • LRP4 (LDL Receptor related Protein 4) antibodies.

• EDrophonium for Diagnosis.

• pyRIDostigmine to get RID of symptoms”).
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