散发型克罗伊茨费尔特-雅各布病患者的睡眠-觉醒紊乱  

Sleep-wake disturbances in sporadic Creutz-feldt-Jakob disease

作　　者：Landolt H.-P Glatzel M. Blttler T. C.L. Bassetti 张嘉靖 

机构地区：[1]Neurologische Universittsklinik,Universittsspitalzürich, Frauenklinikstrasse 26, 8091 Zürich, Switzerland Dr.

出　　处：《世界核心医学期刊文摘（神经病学分册）》2006年第9期27-28,共2页Digest of the World Core Medical Journals:Clinical Neurology

摘　　要：Background: The prevalence and characteristics of sleep-wake disturbances in sporadic Creutzfeldt-Jakob disease (sCJD) are poorly understood. Methods: Seven consecutive patients with definite sCJD underwent a systematic assessment of sleep-wake disturbances, including clinical history, videopolysomnography, and actigraphy. Extent and distribution of neurodegeneration was estimated by brain autopsy in six patients. Western blot analyses enabling classification and quantification of the protease-resistant isoform of the prion protein, PrPSc, in thalamus and occipital cortex was available in four patients. Results: Sleep-wake symptoms were observed in all patients, and were prominent in four of them. All patients had severe sleep EEG abnormalities with loss of sleep spindles, very low sleep efficiency, and virtual absence of REM sleep. The correlation between different methods to assess sleep-wake functions (history, polysomnography, actigraphy, videography) was generally poor. Brain autopsy revealed prominent changes in cortical areas, but only mild changes in the thalamus. No mutation of the PRNP gene was found. Conclusions: This study demonstrates in sporadic Creutzfeldt-Jakob disease, first, the existence of sleep-wake disturbances similar to those reported in fatal familial insomnia in the absence of prominent and isolated thalamic neuronal loss, and second, the need of a multimodal approach for the unambiguous assessment of sleep-wake functions in these patients.Background： The prevalence and characteristics of sleep-wake disturbances in sporadic Creutzfeldt-Jakob disease （sCJD） are poorly understood. Methods： Seven consecutive patients with definite sCJD underwent a systematic assessment of sleep-wake disturbances, including clinical history, videopolysomnography, and actigraphy. Extent and distribution of neurodegeneration was estimated by brain autopsy in six patients. Western blot analyses enabling classification and quantification of the protease-resistant isoform of the prion protein, PrP^Sc, in thalamus and occipital cortex was available in four patients. Results： Sleep-wake symptoms were observed in all patients, and were prominent in four of them. All patients had severe sleep EEG abnormalities with loss of sleep spindles, very low sleep efficiency, and virtual absence of REM sleep. The correlation between different methods to assess sleep-wake functions （history, polysomnography, actigraphy, videography） was generally poor. Brain autopsy revealed prominent changes in cortical areas, but only mild changes in the thalamus. No mutation of the PRNP gene was found. Conclusions： This study demonstrates in sporadic Creutzfeldt-Jakob disease, first,

关 键 词：睡眠-觉醒 散发型 紊乱 病患 罗伊 多导睡眠图 朊蛋白基因 神经退行性变 

分 类 号：R338.22[医药卫生—人体生理学]