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Sleep-wake disturbances in sporadic Creutzfeldt-Jakob disease

From the Institute of Pharmacology & Toxicology (H.-P.L., P.A., I.T.), University of Zürich; Departments of Neuropathology (M.G., A.A.) and Neurology (T.B., C.L.B.), University Hospital, Zürich, Switzerland; Sleep Center and Department of Neurology (C.R., J.M.) and Institute of Neuropathology (J.W.), University Hospital, Bern, Switzerland; Institute of Neuropathology (J.W.); RWTH University, Aachen, Germany; and Institute of Neuropathology (M.G.), University Clinic, Hamburg-Eppendorf, Hamburg, Germany.

Address correspondence and reprint requests to Prof. Claudio L. Bassetti, Neurologische Universitätsklinik, Universitätsspital Zürich, Frauenklinikstrasse 26, 8091 Zürich, Switzerland; e-mail: claudio.bassetti{at}usz.ch

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, 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, 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.

Supported by the Swiss National Science Foundation and the Human Frontiers Science Program.

Disclosure: The authors report no conflicts of interest.

Received June 7, 2005. Accepted in final form January 19, 2006.

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