Abstract:

Objective    To study the clinical features，treatment and prognosis of multiple sclerosis（MS） in children in order to improve the the diagnosis and treatment level. Methods    The clinical data of 84 MS children hospitalized from Nov. 1993 to Dec. 2014 in Department of Pediatrics，Peking University First Hospital were analyzed retrospectively and the follow-ups were summarized. Results    Main clinical manifestations included visual acuity decrease，weakness，headache， fever， encephalopathy and seizure. CSF oligoclonal band was positive in 22/78 cases （28.2%），and CSF myelin-oligodendrocytes glycoprotein antibody was positive in 16/49 cases （32.7%）. Lesions on MRI were frequently observed in subcortical white matter （92.9%） and ventricular white matter （86.9%）， then in cerebellum（47.6%） in brainstem （36.9%）， basal ganglia （29.8%）， thalamus （21.4%）， and corpus callosum （16.7%）. All cases were treated with glucocorticoids at acute stage，combined with intravenous immune globulin （IVIG） in 53 cases （63.1%）. Complete remission was achieved in 69 cases （82.1%） after treatment. The cases were followed-up for （0.7—22.0） years. The average relapse times were （4.71±3.18）. The annual relapse rate was （0.65±0.52） times/year. A total of 61 cases （72.6%） relapsed within the first year after the onset of MS. Fifty-seven cases were followed up to December 2014， and 17/57 cases （12.3%） had movement disorder， and 17/57 cases （29.8%） had visual acuity decrease， and 21/53 （39.6%） had learning disabilities. Conclusion    MS with early onset （younger than 10 years） is not rare. The common presentation symptoms include visual acuity decrease， weakness， headache， fever， encephalopathy and seizure. The positive rate of CSF oligoclonal bands is low. MRI shows the lesions are frequently located in subcortical and ventricular white matter. Glucocorticoids are effective in the acute period. Relapse usually occurrs in the first year after onset. DMT can prolong the relapse interval.Visual acuity decrease and learning disabilities are the common sequelae of the children with MS.

Key words: children, multiple sclerosis, clinical features, treatment

摘要：

目的    探讨儿童多发性硬化（multiple sclerosis，MS）临床特点、治疗及预后，以提高其诊治水平。方法　回顾性分析1993年11月至2014年12月北京大学第一医院儿科84例住院MS患儿临床资料及随访情况进行总结。结果　84例主要临床表现为视力下降、无力、头痛、发热、脑病症状、惊厥。脑脊液寡克隆区带阳性率为28.2%，抗髓鞘少突胶质细胞糖蛋白抗体阳性率为32.7%。头颅磁共振成像（MRI）示92.9%存在皮质下白质脱髓鞘、 86.9%存在脑室旁白质病变、47.6%小脑病变、36.9%脑干病变、29.8%基底节病变、21.4%丘脑病变、16.7%胼胝体病变。急性期均给予糖皮质激素治疗，53例合用大剂量丙种球蛋白，69例首次发作治疗后完全缓解。随访0.7～22.0年，平均复发次数（4.71±3.18）次，平均年复发（0.65±0.52）次/年，61例在首次发病1年内复发。57例随访至2014年12月，12.3%有运动异常，29.8%有视力下降，39.6%有学习障碍。结论　10岁内发病的MS并不少见，常以视力下降、无力、头痛、 发热、脑病、惊厥为首发症状；脑脊液寡克隆区带阳性率低；MRI显示皮质下、脑室旁白质病变多见；急性期糖皮质激素治疗有效，多在1年内复发，疾病修正治疗（DMT）可延长复发间隔，视力下降与学习障碍为其常见的后遗症。

关键词: 儿童, 多发性硬化, 临床特征, 治疗