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KMID : 1035220140060010016
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Korean Journal of Neuromuscular Disorders
2014 Volume.6 No. 1 p.16 ~ p.24
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Treatment of Neuromuscular Junction Disorders
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Kang Sa-Yoon
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Abstract
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Disorders of the neuromuscular junction, such as myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS) and neuromyotonia, constitute an important and treatable class of diseases. These disorders can be of immunological, toxic or genetic origin. MG includes heterogeneous autoimmune disease with a postsynaptic defect of neuromuscular transmission as the common feature. Acetylcholinesterase inhibitors are often the first modality of therapy for MG, but with some caution in patients with MuSK antibodies. Oral corticosteroids are first choice drugs when immunosuppressive drugs are necessary. Plasma exchange and intravenous immunoglobulin are effective for the treatment of MG exacerbations. For patients with non- thymomatous MG, thymectomy is recommended as an option to increase the probability of remission or improvement. Once thymoma is diagnosed, thymectomy is indicated irrespective of MG severity. The treatment of choice for symptom control in LEMS remains 3,4-diaminopyridine. If symptomatic treatment is insufficient, immunosuppressive therapy should be started, usually with a combination of prednisolone and azathioprine. A combination of symptomatic treatment with 3,4-diaminopyridine and immunosuppression, with or without antitumor therapy, is often successful for LEMS patients. Neuromyotonia may improve with plasma exchange and other immunosuppressive treatment. Symptomatic relief may be achieved with antiepileptic drugs, which reduce nerve excitability by blocking sodium channels. For paraneoplastic LEMS and neuromyotonia optimal treatment of the underlying tumor is essential. New biological agents in the form of monoclonal antibodies or fusion proteins offer new hope for target-specific therapy.
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KEYWORD
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Lambert-Eaton myasthenic syndrome, Myasthenia gravis, Neuromuscular junction, Neuromyotonia, Treatment
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