Department of Ophthalmology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Department of Ophthalmology, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Pediatr Neurol. 2018 Oct;87:36-41. doi: 10.1016/j.pediatrneurol.2018.06.007. Epub 2018 Jun 30.
Isolated ocular myasthenia gravis (MG) is sparingly common in children relative to adults, ranging from 71% to 93% of all children with MG.
We aimed to characterize the ocular manifestations and outcomes in children with isolated ocular MG.
Medical records of consecutive 62 subjects less than 15 years of age with ocular MG, were retrospectively reviewed. Demographic data, presenting ocular features, types and variabilities of duction limitation, MG confirmatory tests, types of and responses to treatment, and generalized MG conversion were reviewed.
Mean age at onset and follow-up time were 49 months (range, one to 173 months) and 95 months (range, six to 226 months), respectively. Female-to-male ratio was 1.5:1. Initially, ptosis was found in 60 subjects (96.8%), while duction limitation was observed in 28 subjects (45.2%). Total ophthalmoparesis was the most common type of duction limitation. Variability of duction limitation was found in 68% of subjects during the follow-up. Pyridostigmine alone was the most common medication used (48.4%); ptosis was more responsive to therapy than duction limitation. Conversion to generalized MG occurred in 19.4% of subjects, with a mean interval to conversion of nine months after symptom onset. Most conversions (91.7%) occurred in the first two years.
Ptosis was more responsive to treatment than duction limitation. Thus other treatment modalities, as well as strabismic amblyopia screening, should be considered in children with prolonged duction limitation that is refractory to medication. In contrast with adults, a much lower proportion of children converted to generalized MG. This may explain the higher prevalence of isolated ocular MG among the juvenile population.
与成年人相比，儿童孤立性眼肌型重症肌无力（MG）相对少见，占所有 MG 患儿的 71%至 93%。
我们旨在描述儿童孤立性眼肌型 MG 的眼部表现和结局。
回顾性分析了连续 62 例年龄小于 15 岁的孤立性眼肌型 MG 患儿的病历。回顾了人口统计学数据、眼部表现、眼球运动受限的类型和变化、MG 确诊试验、治疗类型和反应以及全身性 MG 转化。
发病年龄和随访时间的平均值分别为 49 个月（范围，1 至 173 个月）和 95 个月（范围，6 至 226 个月）。女性与男性的比例为 1.5:1。最初，60 例（96.8%）出现上睑下垂，28 例（45.2%）出现眼球运动受限。完全性眼肌麻痹是最常见的眼球运动受限类型。68%的患儿在随访过程中出现眼球运动受限的变化。单独使用吡啶斯的明是最常用的药物（48.4%）；上睑下垂的治疗反应优于眼球运动受限。19.4%的患儿发展为全身性 MG，症状出现后平均 9 个月出现转化。大多数（91.7%）转化发生在最初两年内。
上睑下垂的治疗反应优于眼球运动受限。因此，对于药物治疗无效且眼球运动受限持续时间较长的患儿，应考虑其他治疗方法以及斜视性弱视筛查。与成年人不同，儿童转化为全身性 MG 的比例要低得多。这可能解释了青少年人群中孤立性眼肌型 MG 患病率较高的原因。
