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Autoimmune DiseasesVolume 2011 2011, Article ID 404101, 7 pages

Review ArticleDepartment of Paediatric Neurology, Children-s Hospital, John Radcliffe Hospital, Oxford OX3 9DU, UK

Received 1 June 2011; Accepted 10 August 2011

Academic Editor: Johan A. Aarli

Copyright © 2011 Maria F. Finnis and Sandeep Jayawant. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Myasthenia gravis MG is an autoimmune disease in which antibodies are directed against the postsynaptic membrane of the neuromuscular junction, resulting in muscle weakness and fatigability. Juvenile myasthenia gravis JMG is a rare condition of childhood and has many clinical features that are distinct from adult MG. Prepubertal children in particular have a higher prevalence of isolated ocular symptoms, lower frequency of acetylcholine receptor antibodies, and a higher probability of achieving remission. Diagnosis in young children can be complicated by the need to differentiate from congenital myasthenic syndromes, which do not have an autoimmune basis. Treatment commonly includes anticholinesterases, corticosteroids with or without steroid-sparing agents, and newer immune modulating agents. Plasma exchange and intravenous immunoglobulin IVIG are effective in preparation for surgery and in treatment of myasthenic crisis. Thymectomy increases remission rates. Diagnosis and management of children with JMG should take account of their developmental needs, natural history of the condition, and side-effect profiles of treatment options.





Autor: Maria F. Finnis and Sandeep Jayawant

Fuente: https://www.hindawi.com/



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