Finnis Maria F, Jayawant Sandeep
Department of Paediatric Neurology, Children's Hospital, John Radcliffe Hospital, Oxford OX3 9DU, UK.
Autoimmune Dis. 2011;2011:404101. doi: 10.4061/2011/404101. Epub 2011 Nov 1.
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.
重症肌无力（MG）是一种自身免疫性疾病，其中抗体针对神经肌肉接头的突触后膜，导致肌肉无力和易疲劳。青少年型重症肌无力（JMG）是儿童期的一种罕见病症，具有许多与成人MG不同的临床特征。特别是青春期前儿童，孤立性眼部症状的患病率较高，乙酰胆碱受体抗体的频率较低，缓解的可能性较高。幼儿的诊断可能会因需要与没有自身免疫基础的先天性肌无力综合征相鉴别而变得复杂。治疗通常包括抗胆碱酯酶药物、使用或不使用类固醇节省剂的皮质类固醇，以及新型免疫调节剂。血浆置换和静脉注射免疫球蛋白（IVIG）在手术准备和重症肌无力危象的治疗中有效。胸腺切除术可提高缓解率。JMG患儿的诊断和管理应考虑其发育需求、病情的自然史以及治疗选择的副作用。
