典型克-雅氏病2例并文献回顾  

Creutzfeldt-Jacob disease--two cases and literature review

作　　者：乔琦[1] 王惠娟[1] 张丽苗 岳赞[1] 王炳雷[1] 边鑫[1] 

机构地区：[1]河北医科大学第二医院神经内科,河北石家庄050000

出　　处：《临床荟萃》2017年第4期331-335,共5页Clinical Focus

摘　　要：目的探讨克-雅氏病(Creutzfeldt-Jacob disease,CJD)的发病机制,病因分型,临床表现,脑脊液的特点,核磁共振成像表现,脑电图改变及诊断标准。方法对本院收治的2例CJD患者的临床资料进行分析。结果 CJD临床特征为进行性痴呆、共济失调、肌阵挛。脑电图的周期性尖-慢复合波,核磁共振皮层异常高信号(缎带征)以及脑脊液中的14-3-3蛋白均支持该病的诊断。结论 CJD在脑电图、核磁共振及脑脊液中具有典型的特征。尤其是核磁共振的弥散成像,作为一种无创的检测手段具有较高的敏感度和特异度。Objective To explore the pathogenesis,etiological subtype,clinical manifestation,cerebrospinal fluid（CSF）characters,magnetic resonance imaging（MRI）findings,electroencephalogram（EEG）changes and diagnosis criteria of Creutzfeldt-Jacob disease（CJD）.Methods Analysis was made in the clinical data of two CJD patients from the hospital.Results CJD showed the clinical manifestation predominantly of progressive dementia,ataxia and myoclonus.Occurrence of periodic sharp wave complexes（PSWCs）in EEG,hyper intense signal changes in cortical regions（cortical ribboning）in MRI,and protein 14-3-3in CFS substantiate all supported the diagnosis.ConclusionCJD has typical characters of EEG,MRI and CFS.Specially,diffusion weighted imaging may be a non-invasive screening tool with higher sensitivity and specificity.

关 键 词：脊髓疾病 痴呆 磁共振成像 脑电描记术 脑脊液 

分 类 号：R744[医药卫生—神经病学与精神病学]