Creutzfeld-Jakob病的临床及脑电图特点

A Study of Clinical Syndrome and Electroencephalography in Sporadic Creutzfeld-Jakob Disease

目的探讨克雅(氏)病(Creutzfeld-Jakob disease,CJD)的临床表现及脑电图特点。方法回顾分析10例CJD患者的临床表现及脑电图特点。结果CJD患者以进行性痴呆和肌阵挛最常见,首发症状多为抑郁、失眠、头痛、头晕、记忆力减退及行走不稳。典型脑电图呈阵发周期现象,周期波为高幅尖波、慢波、三相波或多相波,脑电图异常程度随患者病情加重而持续性加重。MRI异常表现为基底节区可见等T1/长T1、长T2信号,双侧基底节对称性钙化及脑皮质萎缩,部分脑脊液蛋白质增高。结论结合典型临床表现,动态脑电图可为CJD的早期临床诊断提供依据。

Objective To study the clinical characteristics and EEG of sporadic Creutzfeld-Jakob disease （CJD）. Methods To review and analyze the clinical manifestation and EEG character of 10 .CJD cases. Results The initial and prominent symptoms include depression, insomnia, headache, dizziness, memory impairment and gait ataxia. The most common symptoms include progressive dementia and myoclonus. The typical EEG exhibits the paroxysmal phenomenia. Periodic wane including high amplitude spike-wane, slow wane, three phase and multiple wane. With the patient＇s condition getting worse, the EEG abnormality is getting worse persistently. MRI has shown abnormal equal T1/long T1, long T2 signal or symmetric bilateral calcified foci in the basal ganglia or atrophy of the cerebral cortex. Proteins in CSF in CJD are elevated in some of the patients. Conclusions On the basis of having the typical manifestation, the character of ambulatory EEG can be considered a foundation for early diagnosis of CJD.