目的探讨小儿脱髓鞘病的临床特点,以提高其诊治水平。方法对本院45例小儿脱髓鞘病的临床特点、辅助检查结果 (包括影像学、肌电图、脑脊液、视觉诱发电位)及治疗方法与疗效进行分析。结果 45例脱髓鞘病患儿中有前驱感染史23例,疫苗接种史1例,多呈急性或亚急性起病。周围性脱髓鞘病多以双下肢或四肢瘫痪为首发;中枢性脱髓鞘病以视力障碍、肢体无力、发热、抽搐、头痛等为首发。周围性脱髓鞘病患儿肌电图多呈神经源性改变,脑脊液多呈蛋白细胞分离;头颅CT/MRI检查:除1例半卵圆区有改变外,余均正常;中枢性脱髓鞘病患儿MRI均显示脱髓鞘改变,表现为颅内不同部位点状或斑片状T2高信号或伴T1低信号;脑电图及脑脊液有不同程度改变,脑脊液白细胞、蛋白及免疫球蛋白升高,4例视觉诱发电位及1例听觉诱发电位异常。结论小儿周围性脱髓鞘病诊断较易,中枢性脱髓鞘病临床表现复杂多变,需通过病史、影像学及实验室检查全面分析。综合治疗预后良好。 Objective To investigate the clinical features of pediatric demyelinating disease in order to improve its diagnosis and treatment. Methods The clinical features, auxiliary examinations (including imaging, EMG, cerebrospinal fluid, visual evoked potentials) and treatment methods and efficacy of 45 children with demyelinating disease in our hospital were analyzed. Results Among the 45 children with demyelinating disease, 23 cases had a history of prodromal infection and 1 case had a history of vaccination, most of them were acute or subacute onset. Peripheral demyelinating disease and more to lower limbs or quadriplegia as the first episode; central demyelinating disease with visual impairment, limb weakness, fever, convulsions, headache, etc. as the first episode. Surgical myoelectric changes in children with peripheral demyelinating mostly neurogenic changes, most of the cerebrospinal fluid were isolated from protein cells; CT / MRI examination: except for 1 case of semi-oval area have changed, the remaining were normal; central demyelination MRI showed demyelinating changes in children with sheath disease, showing different parts of the brain punctate or patchy T2 high signal or with low signal; EEG and cerebrospinal fluid have varying degrees of change, cerebrospinal fluid leukocytes, protein and immunoglobulin Elevated protein, 4 cases of visual evoked potential and 1 case of auditory evoked potential abnormalities. Conclusion The diagnosis of peripheral demyelinating disease in children is relatively easy. The clinical manifestations of central demyelinating disease are complicated and changeable, and the comprehensive analysis must be conducted through history, imaging and laboratory tests. Comprehensive treatment of good prognosis.