眼部症状为突出表现的23例儿童中枢神经脱髓鞘疾病分析

Clinical analysis on 23 cases of children with central nervous demyelinative disease with remarkable ophthalmic symptoms

目的分析小儿中枢神经脱髓鞘疾病(DMD)临床特点,提高对该病的诊断及治疗水平。方法对23例小儿中枢神经DMD的起病特点、前驱感染、临床表现、辅助检查、诊断及治疗等进行回顾性分析。结果本文23例有前驱感染史17例,疫苗接种史3例,呈急性或亚急性起病,均以眼部症状为突出表现,同时或先后出现肢体无力或瘫痪、发热、抽搐及脑神经受损。23例行MRI检查,均呈现异常信号,16例行脑电图检查,12例异常,8例行诱发电位检查,显示视觉和听觉通路损伤。均用皮质激素治疗,15例同时并用大剂量静脉注射免疫球蛋白,均取得显著效果。结论小儿中枢神经DMD临床表现复杂多变,诊断应依据病史、辅助检查,进行综合分析。皮质激素及大剂量免疫球蛋白具有显著疗效,该病大多预后良好。

Objective To explore the clinical characteristics of children with central nervouse demyelinative disease（DMD） and to improve the ability of diagnosis and therapy. Methods Characteristics of onset, presymptoms, clinical manifestations, auxiliary examinations, diagnosis and therapy of 23 chlidren diagnosed with central nervous DMD were analyzed retrospectively. Results Before illness, 17 cases were relevant to infection ,3 cases had vaccine inoculation. Most of the children had acute courses. In the initial stage, ophthalmic symptoms of all cases were marked. Other complicated symptoms were presented, such as somatasthenia or paralysis, fever, convulsion and encephalic nervous lesion. 23 cases showed abnomal signal on MRI. 12 cases showed abnomal among 16 cases EEG examinations. 8 cases evoked potential examinations showed visual and auditory pathway lesion, Steroid was administrated in all cases, 15 cases of them were received higher dose intravenous immunoglobulin（IVIG）. All the cases showed significant improvement in clinic. Conclusion Children with central nervous DMD displays diversified clinical manifestations. By investigation case history combined with auxiliary examinations and aggregate analysis, it can be made a correct diagnosis. Using steroid and higher dose IVIG on treating children DMD has remarkable curative effects, and most of the prognosis is well.