少年慢性炎性脱髓鞘性多发神经病临床分析Analysis of the juvenile with chronic inflammatory demyelinating polyneuropathy
李刚,刘舒鑫,徐军,臧卫周,王琳琳,张宁,杨欢,肖波,梁静慧
摘要(Abstract)：
目的研究少年慢性炎性脱髓鞘性多发神经病(CIDP)的临床特征。方法对14例少年CIDP患者的临床表现、脑脊液检查、肌电图检查及腓肠神经活检结果进行分析。结果本组患者男女比例3.67∶1,发病前有上呼吸道感染5例;首发症状为双下肢远端无力5例,四肢远端麻木无力4例,四肢无力和双下肢远端麻木无力各2例,四肢肌痛1例;患者都有运动障碍,四肢远端运动和感觉同时明显受累11例,肢体远端肌肉萎缩2例;伴肌痛4例;腱反射减弱6例,消失8例;脑神经损害1例;自主神经受累2例;复发1例;发病高峰期Hughes评分平均3.84分;脑脊液检查蛋白细胞分离9例;肌电图示神经源性损害14例;神经活检示髓鞘脱失5例,炎性细胞浸润5例,髓鞘再生2例;激素治疗12例有效。结论本组患者男性多见;首发症状多为对称性双下肢或四肢远端麻木无力;绝大部分有感觉障碍;脑神经和自主神经损害少见;腓肠神经活检有助于诊断;激素治疗有效。
关键词(KeyWords)： 慢性炎性脱髓鞘性多发神经病;少年
基金项目(Foundation):
作者(Author): 李刚,刘舒鑫,徐军,臧卫周,王琳琳,张宁,杨欢,肖波,梁静慧
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