Department of Ophthalmology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA.
Department of Biostatistics, Mayo Clinic and Mayo Foundation, Florida, Minnesota, USA.
Am J Ophthalmol. 2019 Apr;200:242-249. doi: 10.1016/j.ajo.2019.01.004. Epub 2019 Jan 14.
To report the incidence, demographics, and ocular findings of children with myasthenia.
Retrospective cohort study.
The medical records of all children (<19 years) examined at Mayo Clinic with any form of myasthenia from January 1 1966, through December 31, 2015, were retrospectively reviewed.
A total of 364 children were evaluated during the study period, of which 6 children were residents of the Olmsted County at the time of their diagnosis, yielding an annual age- and sex-adjusted incidence of 0.35 per 100 000 <19 years, or 1 in 285 714 <19 years. The incidence of juvenile myasthenia gravis (JMG) and congenital myasthenic syndrome (CMS) was 0.12 and 0.23 per 100 000, respectively. Of the 364 study children, 217 (59.6%) had JMG, 141 (38.7%) had CMS, and 6 (1.7%) had Lambert-Eaton syndrome, diagnosed at a median age of 13.5, 5.1, and 12.6 years, respectively. A majority of the JMG and CMS patients had ocular involvement (90.3% and 85.1%, respectively), including ptosis and ocular movement deficits. Among children with at least 1 year of follow-up (JMG; median, 7.1 years, CMS; median, 7.0 years), improvement was seen in 88.8% of JMG patients (complete remission in 31.3%) and in 58.3% of CMS patients.
Although relatively rare, myasthenia gravis in children has 2 predominant forms, CMS and JMG, both of which commonly have ocular involvement. Improvement is more likely in children with the juvenile form.
报告患有重症肌无力的儿童的发病率、人口统计学和眼部发现。
回顾性队列研究。
回顾性分析了 1966 年 1 月 1 日至 2015 年 12 月 31 日期间在梅奥诊所接受任何形式重症肌无力检查的所有<19 岁儿童的病历。
在研究期间共评估了 364 名儿童，其中 6 名儿童在诊断时是奥姆斯特德县的居民，这使得每 100000 名<19 岁儿童的年龄和性别调整后的年发病率为 0.35，即每 285714 名<19 岁儿童中有 1 名。青少年型重症肌无力（JMG）和先天性肌无力综合征（CMS）的发病率分别为 0.12 和 0.23。在 364 名研究儿童中，217 名（59.6%）患有 JMG，141 名（38.7%）患有 CMS，6 名（1.7%）患有 Lambert-Eaton 综合征，中位年龄分别为 13.5 岁、5.1 岁和 12.6 岁。大多数 JMG 和 CMS 患者有眼部受累（分别为 90.3%和 85.1%），包括上睑下垂和眼球运动障碍。在至少有 1 年随访的儿童中（JMG；中位数，7.1 年，CMS；中位数，7.0 年），JMG 患者中有 88.8%（31.3%完全缓解）和 CMS 患者中有 58.3%有改善。
尽管相对罕见，但儿童重症肌无力有 2 种主要形式，CMS 和 JMG，两者都常见眼部受累。幼年型患儿更有可能改善。
