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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。
Objective To investigate the clinical features, treatment and prognosis of children with multiple sclerosis (MS) so as to improve the diagnosis and treatment of multiple sclerosis. Methods The clinical data and follow-up of 84 inpatients with pediatric MS at Peking University First Hospital from November 1993 to December 2014 were retrospectively analyzed. Results 84 cases of the main clinical manifestations of vision loss, weakness, headache, fever, encephalopathy symptoms, convulsions. Cerebrospinal fluid oligoclonal band positive rate was 28.2%, anti-myelin oligodendrocyte glycoprotein antibody positive rate was 32.7%. Head MRI showed 92.9% of subcortical demyelination, 86.9% of IV lesions, 47.6% of cerebellar lesions, 36.9% of brain stem lesions, 29.8% of basal ganglia lesions, and 21.4% of thalamic lesions with 16.7% Corpus callosum lesions. Corticosteroids were given in the acute phase, and 53 cases were given high-dose gamma globulin. The complete response was relieved in 69 cases after the first episode of treatment. The follow-up ranged from 0.7 to 22.0 years. The average number of recurrence was (4.71 ± 3.18) times and the average annual recurrence was (0.65 ± 0.52) times / year. Sixty-one patients relapsed within one year of first onset. 57 cases were followed up to December 2014, 12.3% had motor abnormalities, 29.8% had vision loss, 39.6% had learning disabilities. Conclusion The incidence of MS within 10 years of age is not uncommon, often with decreased vision, weakness, headache, fever, encephalopathy, seizures as the first symptom; cerebrospinal fluid oligoclonal band positive rate; MRI showed cortical, ventricular white matter lesions more common; Acute phase of glucocorticoid treatment is effective, more than 1 year recurrence, disease correction therapy (DMT) can extend the recurrence interval, vision loss and learning disabilities as its common sequelae.