Ortiz Steve, Borchert Mark
Department of Ophthalmology, Vision Center at Childrens Hospital Los Angeles, University of Southern California, Keck School of Medicine, Los Angeles, California, USA.
Ophthalmology. 2008 Jul;115(7):1245-1248.e1. doi: 10.1016/j.ophtha.2007.10.022. Epub 2007 Dec 26.
To review the presenting signs, therapeutic interventions, and clinical outcomes of purely ocular myasthenia gravis in a preadolescent population treated primarily with pyridostigmine bromide.
Retrospective case series.
Twenty-one consecutive patients younger than 12 years with purely ocular myasthenia gravis at initial presentation.
The clinical charts were reviewed retrospectively.
Time to generalization, stabilization, or resolution; prism cover test results; and visual acuity.
Median age at onset was 26 months. The mean duration of follow-up was 6.5 years (range, 2-15). Presenting signs included ptosis (95%), strabismus (76%), limitation of ductions (17%), and Cogan's lid twitch (76%). The most common form of strabismus was exotropia. Pyridostigmine monotherapy was the initial treatment for all patients. Corticosteroids were added to the therapy for 6 patients. Generalization to systemic disease occurred in 3 patients. Two of those required intravenous immunoglobulin and thymectomy. Complete resolution off of medical therapy occurred in 4 patients. All patients had stabilization of the ocular motor deficits regardless of treatment. Eleven patients were treated for amblyopia; 2 had residual amblyopia.
Ocular myasthenia in preadolescent children generally presents before age 5 and is clinically distinguished from the disease as it affects adults. Although the presenting signs of strabismus, ptosis, and Cogan's lid twitch are common in children or adults, the response to treatment and eventual outcomes differ. Most children can be safely treated with pyridostigmine alone. Generalization to systemic disease occurs at a much lower rate than in adults. Ocular manifestations stabilize in all children and completely resolve in some.
回顾主要采用溴吡斯的明治疗的青春期前单纯眼型重症肌无力患者的临床表现、治疗干预措施及临床结局。
回顾性病例系列研究。
21例初诊时年龄小于12岁的单纯眼型重症肌无力患者。
对临床病历进行回顾性分析。
病情泛化、稳定或缓解的时间；三棱镜遮盖试验结果；以及视力。
发病的中位年龄为26个月。平均随访时间为6.5年（范围2 - 15年）。临床表现包括上睑下垂（95%）、斜视（76%）、眼球运动受限（17%）和科根眼睑抽搐（76%）。最常见的斜视类型是外斜视。所有患者初始治疗均采用溴吡斯的明单药治疗。6例患者在治疗中加用了皮质类固醇。3例患者病情泛化为全身性疾病。其中2例需要静脉注射免疫球蛋白和胸腺切除术。4例患者在停药后完全缓解。无论采用何种治疗，所有患者的眼球运动功能障碍均得到稳定。11例患者接受了弱视治疗；2例仍有残余弱视。
青春期前儿童的眼型重症肌无力通常在5岁前发病，在临床上与成人患该病有所不同。尽管斜视、上睑下垂和科根眼睑抽搐等临床表现儿童和成人都较为常见，但治疗反应和最终结局有所不同。大多数儿童仅用溴吡斯的明即可安全治疗。病情泛化为全身性疾病的发生率远低于成人。所有儿童的眼部表现均得到稳定，部分儿童完全缓解。
