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1、中国神经精神疾病杂志2008年第34卷第12期715·论著·儿童慢性炎症性脱鞘性多神经病的临床和病理改变特点3333333△3栾兴华郑日亮陈彬常杏芝熊辉吕俊兰袁云【摘要】目的探讨儿童慢性炎症性脱鞘性多神经病(chronicinflammatorydemyelinatingpolyneuropathy,CI2DP)的临床及病理改变特点。方法根据欧洲神经肌肉病中心修订的儿童CIDP诊断标准诊断的10例17岁以下患者,收集其临床资料,进行周围神经电生理以及腓肠神经的病理检查。结果所有患者主要表现为肢体无力,分别有4例和3例出现四肢感觉减退和颅神经损害。9例有脑脊液蛋白细胞分离
2、现象。10例均出现运动或感觉神经传导速度减慢及远端潜伏期延长,9例患者的动作电位波幅降低。所有患者的有髓神经纤维出现轻-重度减少,其中3例患者的纤维脱失程度在不同束间存在差异,6例患者以脱髓鞘为主;3例以轴索损害为主。1例患者仅出现轻微改变。9例患者存在炎细胞浸润。结论儿童CIDP以肢体无力为主。部分患者以轴索损害为主,神经纤维脱失程度可以存在束间差异。【关键词】慢性炎症性脱鞘性多神经病周围神经病儿童【中图分类号】R744.5【文献标识码】AClinicalandpathologicalfeaturesinthechildhoodchronicinflammatoryd
3、emyelinatingpolyneuropathy1LUANXinghua,ZHENGRiliang,CHENBin,CHANGXingzhi,XIONGHui,LVJunlan,YUANYan.DepartmentofNeurologyandLaboratoryofNewropathology,FirstHospitalofPekingUniversity,8XishikuSt.XichengDistrict,Beijing100034,China.Tel:010-6655112-2588.【Abstract】ObjectiveTostudytheclinical,
4、electrophysiologicalandpathologicalfeaturesinthechildhoodchron2icinflammatorydemyelinatingpolyneuropathy(CIDP).MethodsTenpatientswhoseageatonsetwasbetween2～17yearsoldwerediagnosedbytheEuropeanNeuromuscularCentercriteriaforCIDP.Theclinicaldatawerecollectedandelectrophysiologicalexaminatio
5、naswellassuralnervebiopsywereperformedinallpatients.ResultsThemainclinicalsymptomswereproximalweaknessinallcases,amongthem,4casespresentedwithsensorylossintheextremitiesand3ca2seswithmultiplecranialnervepalsy.Cerebrospinalfluidtestshowedalbumino2cytologicaldissociationin9cases.Decreasein
6、motorandsensorynerveconductionvelocitieswithprolongeddistallatencypresentedin10casesanddecreaseinampli2tudeofcompoundactivepotentialin9cases.Mildtoseverelossofmyelinatedfibersappearedinallcasesandfiberlossa2mongthefasciculesvariedmarkedlyin3cases.Sixcasesmainlypresentedwithdemyelination,
7、3caseswithmarkedWalle2riandegeneration,1casewithmildmorphologicalchangesand9casesinflammatoryinfiltrate.ConclusionsMotorweak2nessisthemajorsymptomofchildhoodCIDP.Axonallesionsarethemainmorphologicalchangesinsomepatientsandsub2stantialvariabilityofmyelinatedfiberslossexist
