Sleep and temperature rhythms in two sisters with P102L Gerstmann-Straussler-Scheinker (GSS) disease

被引：9

作者：

Provini, Federica ^{[1
]}

Vetrugno, Roberto ^{[1
]}

Pierangeli, Giulia ^{[1
]}

Cortelli, Pietro ^{[1
]}

Rizzo, Giovanni ^{[1
]}

Filla, Alessandro ^{[2
]}

Strisciuglio, Caterina ^{[2
]}

Gallassi, Roberto ^{[1
]}

Montagna, Pasquale ^{[1
]}

机构：

[1] Univ Bologna, Dipartimento Sci Neurol, I-40123 Bologna, Italy

[2] Univ Naples Federico 2, Dept Neurol Sci, Naples, Italy

来源：

SLEEP MEDICINE | 2009年 / 10卷 / 03期

关键词：

Gerstmann-Straussler-Scheinker; Prion diseases; Sleep; Circadian rhythm; Polysomnography; Neurodegenerative diseases; MULTIPLE-SYSTEM ATROPHY; CREUTZFELDT-JAKOB-DISEASE; REM-SLEEP; BEHAVIOR DISORDER; NEURODEGENERATIVE DISEASE; PARKINSONS-DISEASE; INSOMNIA; ASSOCIATION;

D O I：

10.1016/j.sleep.2008.03.004

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Background Sleep disorders are increasingly recognized in the symptomatology of many neurodegenerative diseases. Gerstmann-Straussler-Scheinker (GSS) disease is a hereditary prion disease featuring cerebellar ataxia, akinetic parkinsonism, pyramidal signs and cognitive decline. Methods: We performed a polysomnographic study (PSG) of sleep and body core temperature (BcT degrees) in two sisters with GSS. Results: Our study showed protracted nocturnal awakenings, reduced sleep efficiency and brief daytime naps but also qualitatively preserved slow-wave and REM sleep and substantially normal arousal and periodic limb movements in sleep indices and BcT degrees rhythm. Conclusions: These findings conflict with those in multiple system atrophy and other prion diseases such as fatal familial insomnia, which enter the differential diagnosis of GSS and are characterized by prominently disrupted sleep-wake and BcT degrees cycles. (C) 2008 Elsevier B.V. All rights reserved.

引用

页码：374 / 377

页数：4

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