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Ann Child Neurol > Volume 25(1); 2017 > Article
Journal of the Korean Child Neurology Society 2017;25(1):1-8.
DOI: https://doi.org/10.26815/jkcns.2017.25.1.1    Published online March 30, 2017.
Subgroup Classification and Therapeutic Guidance for Myasthenia Gravis.
Yun Jin Lee
Department of Pediatrics, Pusan National University Children's Hospital, Pusan National University School of Medicine, Yangsan, Korea. jinnyeye@hanmail.net
Myasthenia gravis (MG) is a chronic autoimmune disease of neuromuscular blockade, characterized by muscle weakness and fatigue, and is associated with the production of autoantibodies against the skeletal muscle acetylcholine receptor, muscle-specific kinase (MuSK), low-density lipoprotein receptor-related protein 4 (LRP4), and other muscle endplate proteins. In addition, MG may be classified according the location of the affected muscles (ocular vs. generalized), patient age at symptom onset, and thymic pathology. Subgroup classification based on serum antibodies and clinical features include early-onset, late-onset, thymoma-associated, MuSK and LRP4 antibody-negative, and ocular forms of MG, and can help with therapeutic decisions and prognosis. Pyridostigmine is the chosen symptomatic treatment. For patients who do not adequately respond to symptomatic therapy, corticosteroids, other immunomodulating agents, and thymectomy are the first-line immunosuppressive treatments. The treatment of MG is highly individualized and depends on the age of the patient, the type and severity of the disease, and the pace of progression.
Key Words: Myasthenia gravis, Child, Adult, Therapy, Thymectomy
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