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散发性和医源性克雅氏病的系列脑电图检查结果

Serial EEG findings in sporadic and iatrogenic Creutzfeldt-Jakob disease.

作者信息

Wieser Heinz Gregor, Schwarz Urs, Blättler Thomas, Bernoulli Christoph, Sitzler Matthias, Stoeck Katharina, Glatzel Markus

机构信息

Department of Neurology, Zürich University Hospital, Zurich, Switzerland.

出版信息

Clin Neurophysiol. 2004 Nov;115(11):2467-78. doi: 10.1016/j.clinph.2004.05.032.

DOI:10.1016/j.clinph.2004.05.032
PMID:15465434
Abstract

OBJECTIVE

To study temporal and spatial development of EEG patterns in sporadic and iatrogenic Creutzfeldt-Jakob disease patients.

METHODS

Temporal and spatial development of EEG patterns in 4 patients with sporadic Creutzfeldt-Jakob disease and 2 patients with iatrogenic Creutzfeldt-Jakob disease due to implantation of contaminated brain depth electrodes were investigated. A total of 56 EEGs were analyzed, over time spans ranging from 1272 to 3 days prior to death.

RESULTS

Frontal intermittent rhythmical delta activity (FIRDA) was seen at early timepoints in 4/6 patients and might represent an early EEG pattern that is associated, with human prion diseases. EEG patterns associated with CJD are sensitive to midazolam. Initial EEG changes were seen at the site of prion exposure in iatrogenic Creutzfeldt-Jakob disease patients, before they could be observed at distant sites, suggesting that prion disease was initiated at the site of prion exposure.

CONCLUSIONS

Serial EEG recordings are a valuable tool not only in the early diagnosis of sporadic CJD, but also in the determination of prion exposure in iatrogenic Creutzfeldt-Jakob disease.

SIGNIFICANCE

FIRDA occur at an early stage of CJD and are progressively replaced by the classical PSWC. The EEG patterns of CJD are sensitive to midazolam. The initial EEG changes in iatrogenic CJD are seen at the site of prion exposure.

摘要

目的

研究散发性克雅氏病(CJD)和医源性克雅氏病患者脑电图(EEG)模式的时空发展。

方法

对4例散发性克雅氏病患者和2例因植入受污染脑深部电极导致医源性克雅氏病的患者的EEG模式的时空发展进行了研究。共分析了56份EEG,时间跨度为死亡前1272天至3天。

结果

4/6例患者在早期出现额叶间歇性节律性δ活动(FIRDA),这可能代表了一种与人类朊病毒病相关的早期EEG模式。与CJD相关的EEG模式对咪达唑仑敏感。医源性克雅氏病患者在朊病毒暴露部位出现了最初的EEG变化,随后才在远处观察到,这表明朊病毒病始于朊病毒暴露部位。

结论

连续EEG记录不仅是散发性CJD早期诊断的有价值工具,也是医源性克雅氏病中朊病毒暴露判定的有价值工具。

意义

FIRDA出现在CJD的早期阶段,并逐渐被经典的周期性同步放电(PSWC)所取代。CJD的EEG模式对咪达唑仑敏感。医源性CJD最初的EEG变化出现在朊病毒暴露部位。

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