Neurology » Muscular problems

Myasthenia Gravis

Myasthenia gravis (MG) is a primary disorder of neuromuscular transmission. The usual cause is an acquired immunological abnormality, but some cases result from genetic abnormalities at the neuromuscular junction. Thymic abnormalities are clearly associated with myasthenia gravis but the nature of the association is uncertain. Ten per cent of patients with myasthenia gravis have a thymic tumour and 70% have hyperplastic changes (germinal centres) that indicate an active immune response. The most common age at onset is the second and third decades in women and the seventh and eighth decades in men. patients present with specific muscle weakness. Ocular motor disturbances, ptosis or diplopia, are the initial symptom of myasthenia gravis in two-thirds of patients. Oropharyngeal muscle weakness, difficulty chewing, swallowing, or talking, is the initial symptom in one-sixth of patients. Diagnosis is by edrophonium Chloride (Tensilon) Test, detection of serum antibodies that bind human AChR and EMG, Cholinesterase Inhibitorscause considerable improvement in some patients and little to none in others. Strength rarely returns to normal. Pyridostigmine bromide and neostigmine bromide are the most commonly used ChE inhibitors. Thymectomy is recommended for most patients with myasthenia gravis. The maximal favourable response generally occurs 2 to 5 years after surgery.

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