59. Myasthenia Gravis
Definition
Myasthenia gravis is an acquired autoimmune disorder of neuromuscular function marked by fatigue and exhaustion of the muscular system. Fluctuations in severity of muscular atrophy accompany periods of increased or decreased activity.
Incidence
Myasthenia gravis is not very common. It is estimated to occur at a rate of 2:1,000,000.
Etiology
Myasthenia gravis is caused by the presence of antibodies against acetylcholine receptors at the neuromuscular junction of skeletal muscles; 80% to 90% of myasthenia gravis patients have antibodies against acetylcholine receptors. Tolerance to acetylcholine receptors by the immune system is somehow lost, but the mechanism is not clear. Myasthenia gravis is considered to be a B-cell–mediated disease process; however, T-cells’ contribution to the process is becoming increasingly more evident. T-cell immunity is dominated by the thymus, and myasthenia gravis patients frequently have thymic hyperplasia, thymoma, or other thymus abnormalities.
Signs and Symptoms
• Acute respiratory failure
• Aspiration
• Difficulty chewing
• Difficulty swallowing
• Horizontal smile
• Hyperthyroidism
• Lupus
• Mask-like facies
• Nasal regurgitation of food and liquids
• Ophthalmoplegia
• Proptosis (exophthalmos)
• Ptosis
• Rheumatoid arthritis
• Scleroderma
Myasthenia Gravis Foundation of America Clinical Classifications
Class I
• Any ocular muscle weakness
• May have weakness of eye closure
• All other muscle strength is normal
Class II
• Mild weakness affecting other than ocular muscles
• May have ocular muscle weakness of any severity
Class IIa
• Predominately affecting limb or axial muscles or both
• May also have lesser involvement of oropharyngeal muscles
Class IIb
• Predominately affecting oropharyngeal or respiratory muscles or both
• May also have lesser or equal involvement of limb or axial muscles or both
Class III
• Moderate weakness affecting other than ocular muscles
• May also have ocular muscle weakness of any severity
Class IIIa
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