Myopathic syndrome in patients with Myasthenia gravis in childhood
Keywords:
myasthenia gravis, myopathic syndrome, thymectomyAbstract
Myasthenia gravis (MG) is rare autoimmune disease affecting neuromuscular junction transmission. MG is characterized by muscle weakness that worsens with activity and fluctuates over the course of the day. MG is termed ocular MG when weakness is exclusive to the eyelids and extraocular muscles, and generalized MG when weakness extends beyond these ocular muscles.
We present three children diagnosed with juvenile myasthenia gravis-generalized form. The diagnosis is based on clinical history and neurologic examination and confirmed by electrodiagnostic testing and the presence of serum autoantibodies to the skeletal muscle nicotinic acetylcholine receptor (AChR) and neuro-imaging data for thymic hyperplasia. Corticosteroids have been administered to the three chidren, and thymectomy was performed in both patients. Three patients developed myopathic syndrome several years after the diagnosis of myasthenia gravis, confirmed by electromyography.
Myasthenia gravis is an autoimmune disease due to serum circulating antibodies against certain structures of the neuromuscular junction. In patients with long-term course of disease and resistant to therapy, myopathic syndrome confirmed by electrophysiological methods is possible.
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