前言目的Creutzfeldt-Jakob病（CJD）临床少见,本文旨在提高临床医生对此病的认识。方法总结复旦大学附属华山医院诊断为CJD的患者11例,行脑电图、头部MRI、DWI、头部CT、CSF14-3-3蛋白检查。结果主要临床症状和体征有进行性痴呆、共济失调、肌阵挛、精神行为异常、锥体束征、视力下降、锥体外系征。脑电图均有异常,9例出现周期性三相波。5例MRI弥散加权像中3例双侧皮质显示异常高信号,1例示基底节及皮质异常高信号,4例行脑脊液14-3-3蛋白检查,1例阳性。结论CJD临床表现以急速发展的进行性痴呆和肌阵挛最具特征性,脑电图、DWI、CSF14-3-3是诊断本病的重要检查手段。
ObjectiveTodeepenthedoctor'sunderstandingofCreutzfeldt-JakobDisease（CJD）,whichisscarceinclinic.MethodsTosumup11casesofCJDpatientsdiagnosedbyHuashanhospitalattachedtoFudanUniversity,someexaminationshadbeendoneincludingelectroencephalogram（EEG）,headMRI,DWI,headCTandCSF14-3-3proteintest.ResultThemajorclinicalsymptomandphysicalsignofCJDwereprogressivedementia,incoordination,myoclonia,vigor-activityabnormal,conebunchsign,visiondecline,outerconesign.TheE...
