3例很可能克雅脑病患者的临床表现及脑电图动态分析

Clinical manifestation and EEG dynamic analysis in 3 patients with probable Creutzfeldt-Jakob disease

目的：分析克雅脑病（CJD）患者的临床表现、病程及脑电图的动态变化。方法：回顾性分析我院3例诊断为很可能CJD的患者，行脑电图、头部MRI、CSF14-3—3蛋白检查。结果：主要临床表现为进行性痴呆、共济失调、肌阵挛、精神行为异常、锥体外系征及缄默等。3例脑电图均有弥漫性异常，均出现典型周期性三相波，1例在视频脑电图监测中发现早期周期性三相波。2例行脑脊液14—3—3蛋白检查，1例阳性。结论：对中年以上起病，快速进展的痴呆，且合并有注意力不集中、记忆力减退、性格改变、肌阵挛、共济失调、视力障碍，以及进行性精神障碍患者，应高度怀疑CJD的可能。脑电图、MRI以及脑脊液14—3—3蛋白的检测，特别是视频脑电图监测有可能更有利于发现早期的三相波，有助于对可疑病例进行早期的诊断。

Objective： To analyze clinical manifestation, course and EEG dynamic change in patients with creutzfeldt-Jakob disease（CJD）. Methods： Retrospective analysis of 3 cases in our hospital which were diagnosed probable CJD, and the examinations were taken including EEG, head MRI and CSF 14-3-3 protein test. Results：The main clinical manifestations of CJD were progressive dementia, ataxia, myoclonus,mental-behavior disorder,extrapyra-midal sign,mutrsm and so on. The EEG results were all diffused abnormality, 3 cases showed periodic three phase waves, and one case showed early periodic three-phase waves in V-EEG. CSF 14-3-3 protein tests were positive in 1 of the 2 cases. Conclusion： In patients with middle-aged onset, rapidly progressive dementia, combined with concentration or memory loss, personality changes, myoclonus, ataxia, visual impairment, as well as mental disorder,they should be highly suspected as CJD. Detection of EEG , head MRI and CSF 14-3-3 protein are important methods ,and in particular, V-EEG monitoring may be more conducive to detection of early three-phase waves, and the early diagnosis of CJD in suspected cases.