双语Multifocalmotor

译者

王力群、拱忠影、薛萍、邱晓峰、

崔兴华、赵思佳、范秉林

文献审校：张慜

文献提供：林宏

李神经文献翻译中心出品

第一部分

AbstractMultifocalmotorneuropathy(MMN)isarareinflammatoryneuropathycharacterizedbyslowlyprogressive,asymmetricdistallimbweaknesswithoutsensoryloss.TheclinicalpresentationofMMNmaymimicamyotrophiclateralsclerosis,othervariantsofmotorneurondisease,orchronicinflammatorydemyelinatingpolyneuropathywithasymmetriconset.Differentiationisimportant,asthesediseasesdifferinprognosisandtreatment.TheelectrophysiologicalfindingofconductionblockintheabsenceofabnormalitiesinsensorynervesisthehallmarkofMMN,butcanbedifficulttodetect.Intravenousimmunoglobulinisefficaciousinmostpatients,butlong-termmaintenancetherapydoesnotpreventslowlyprogressiveaxonaldegeneration.Moreover,cyclophosphamide,althougheffective,hassubstantialadverseeffects,andtheefficacyofotherimmunosuppressivedrugs,includingrituximab,isnotestablished.TheunderlyingpathologicalmechanismsofMMNareunclear,butIgMautoantibodiesagainstthegangliosideGM1maycausechangesinnodalandperinodalstructuresthat







































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