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儿童多发性硬化84例临床研究

发布时间:2018-05-09 05:57

  本文选题:儿童 + 多发性硬化 ; 参考:《中国实用儿科杂志》2016年01期


【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。
[Abstract]:Objective to investigate the clinical features, treatment and prognosis of multiple sclerosis in children. Methods from November 1993 to December 2014, the clinical data and follow-up data of 84 children with MS in pediatrics of Peking University first Hospital were analyzed retrospectively. Results the main clinical manifestations of 84 cases were visual acuity, weakness, headache, fever, encephalopathy and convulsion. The positive rate of oligoclonal bands in cerebrospinal fluid and anti-myelin oligodendrocyte glycoprotein antibody was 28.2and 32.7g respectively. Magnetic resonance imaging (MRI) showed that 92.9% had subcortical white matter demyelination and 86.9% had ventricular collateral leukoencephalopathy 47.6% cerebellar lesions and 29.8% basal ganglia lesions and 21.4% thalamic lesions and 16.7% corpus callosum lesions. In the acute phase, 53 cases were treated with glucocorticoid and 69 cases with high dose gamma globulin were completely relieved after the first attack treatment. During the follow-up period from 0.7 to 22.0 years, the average recurrence times were 4.71 卤3.18 times, and the average annual recurrence frequency was 0.65 卤0.52 times per year. 57 cases were followed up to 12.3% of the patients with motor abnormalities until December 2014. 39.6% of the patients had impaired visual acuity. Conclusion MS within the age of 10 years is not uncommon. The first symptoms are vision loss, weakness, headache, fever, encephalopathy, convulsion, and low positive rate of oligoclonal bands in cerebrospinal fluid (CSF). The treatment of glucocorticoid in acute stage was effective, most of them recurred within one year, the disease correction therapy could prolong the recurrence interval, the visual acuity and learning disability were the common sequelae.
【作者单位】: 北京大学第一医院儿科;武汉市儿童医院神经内科;
【分类号】:R744.51

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