散发型克雅病临床、脑电图及影像学研究

Clinical manifestations and EEG, MRI findings of sporadic Creutzfeldt-Jakob disease

目的 探讨散发型克雅病 （sCJD）的临床、脑电图（EEG）及影像学特点.方法 分析 12 例sCJD 患者的临床表现、EEG、影像学特点.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、锥体外系征.EGG检查12例均有异常,9例出现周期性三相波.12例MRI弥散加权（DWI）像中3 例皮质显示异常高信号,8例示皮质及基底节异常高信号,1例示基底节异常高信号.结论 sCJD临床表现以快速发展的进行性痴呆和肌阵挛最具特征性,EGG、DWI是诊断本病的重要检查手段.

Objective To review the clinical feature, electroencephalography （EEG） and MRI findings of sporadic Creutzfeldt-Jakob disease （sCJD）. Methods The clinical feature, EEG and MRI of 12 sCJD cases were analyzed retrospectively. Results The major clinical symptom and physical sign of sCJD were progressive dementia, ataxia, myocionus, mental and behavioral abnormalities, pyramidal sign, extrapyramidal sign. The EEG results were all abnormal, 9 cases showed periodic triphasic wave. In MRI diffusion-weighted image, 3 cases showed abnormal hyperintense signals in cerebral cortex; 8 cases showed abnormal hyperintense signals in cerebral cortex and basal ganglia; 1 case showed abnormal hyperintense signals in basal ganglia. Conclusion Rapidly progressive dementia and myoclonus are the main characters of sCJD, EEG and DWI are the major diagnostic methods for sCJD.