Myasthenia gravis in children: a longitudinal study
Myasthenia gravis in children: a longitudinal study
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DOI：
10.1111/j.1600-0404.2006.00646.x
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发表时间：
2006-08-01
影响因子：
3.5
通讯作者：
Rao, S.
中科院分区：
文献类型：
--
作者：
Ashraf, V. V.;Taly, A. B.;Rao, S.
背景：幼年型重症肌无力（JMG）是一种少见的疾病。与成人不同，儿童重症肌无力（MG）的临床特征和结局尚未得到充分研究。患者和方法：77例MG患者的病例记录谁是15岁或以下的疾病发作，在34年的时间内在印度班加罗尔的国家精神卫生和神经科学研究所进行了评估，进行了审查。他们的临床特征和对治疗的反应与290例15岁以后发病的MG患者进行了比较。结果：发病时的中位年龄为8岁，平均随访时间为6.2年（范围6个月至25年）。在介绍，30%的患者有眼肌无力，其余的全身性疾病。21例患者（27%）在整个过程中的疾病局限于眼肌，3肢带肌无力。家族性肌无力较成人发病多见，10例有阳性家族史。与成人不同，这些患者都没有相关的自身免疫性疾病。52例患者（67%）接受皮质类固醇，5例患者加用硫唑嘌呤。11例患者行胸腺切除术，其中6例年龄在15岁以下。胸腺组织学正常的一个，表现为增生的八个和胸腺瘤。4例患者出现危象。在随访结束时，25例患者无症状，28例部分改善，9例保持不变或恶化，2例死亡。10例患者获得完全稳定缓解。结论：本研究显示了JMG的一些独特的特征，如眼肌无力的频率较高，良性过程，较好的长期结果和缺乏胸腺瘤和其他自身免疫性疾病的关联。
Background: Juvenile myasthenia gravis (JMG) is an uncommon disease. Unlike adults, clinical characteristics and outcomes of myasthenia gravis (MG) are not well studied in children. Patients and methods: Case records of 77 patients with MG who were 15 years of age or less at disease onset, evaluated over a period of 34 years at the National Institute of Mental Health and Neurosciences, Bangalore, India, were reviewed. Their clinical characteristics and response to therapy was compared with 290 patients with MG onset after 15 years of age. Results: Median age at onset was 8 years and mean period of follow-up was 6.2 years (range 6 months to 25 years). At presentation, 30% of patients had ocular myasthenia and the rest had generalized disease. Twenty-one patients (27%) had disease confined to ocular muscles throughout the course and three had limb girdle myasthenia. Familial myasthenia was more common than adult onset disease, 10 patients had positive family history. Unlike adults, none of the patients had associated autoimmune disease. Fifty-two patients (67%) received corticosteroids, and azathioprine was added in five patients. Thymectomy was performed in 11 patients, six below the age of 15 years. Thymic histology was normal in one and showed hyperplasia in eight and thymoma in one. Four patients had crisis. At the end of follow-up, 25 patients were asymptomatic, 28 had partial improvement, and nine remained unchanged or worsened and two died. Ten patients achieved complete stable remission. Conclusions: This study shows some distinctive characteristics of JMG, such as higher frequency of ocular myasthenia, benign course, better long-term outcome and lack of association of thymoma and other autoimmune disorders.