Sleep-wake disturbances in sporadic Creutzfeldt-Jakob disease

被引:51
|
作者
Landolt, HP
Glatzel, M
Blättler, T
Achermann, P
Roth, C
Mathis, J
Weis, J
Tobler, I
Aguzzi, A
Bassetti, CL
机构
[1] Univ Zurich, Inst Pharmacol & Toxicol, Zurich, Switzerland
[2] Univ Hosp, Dept Neuropathol, Zurich, Switzerland
[3] Univ Hosp, Dept Neurol, Zurich, Switzerland
[4] Univ Hosp, Sleep Ctr, Bern, Switzerland
[5] Univ Hosp, Dept Neurol, Bern, Switzerland
[6] Univ Hosp, Inst Neuropathol, Bern, Switzerland
[7] RWTH Univ, Inst Neuropathol, Aachen, Germany
[8] Univ Clin, Inst Neuropathol, Hamburg, Germany
关键词
D O I
10.1212/01.wnl.0000210445.16135.56
中图分类号
R74 [神经病学与精神病学];
学科分类号
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, video-polysomnography, 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.
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收藏
页码:1418 / 1424
页数:7
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