TY - JOUR
T1 - Sleep and temperature rhythms in two sisters with P102L Gerstmann-Sträussler-Scheinker (GSS) disease
AU - Provini, Federica
AU - Vetrugno, Roberto
AU - Pierangeli, Giulia
AU - Cortelli, Pietro
AU - Rizzo, Giovanni
AU - Filla, Alessandro
AU - Strisciuglio, Caterina
AU - Gallassi, Roberto
AU - Montagna, Pasquale
PY - 2009/3
Y1 - 2009/3
N2 - Background: Sleep disorders are increasingly recognized in the symptomatology of many neurodegenerative diseases. Gerstmann-Sträussler-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°) 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° 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° cycles.
AB - Background: Sleep disorders are increasingly recognized in the symptomatology of many neurodegenerative diseases. Gerstmann-Sträussler-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°) 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° 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° cycles.
KW - Circadian rhythm
KW - Gerstmann-Sträussler-Scheinker
KW - Neurodegerneative diseases
KW - Polysomnography
KW - Prion diseases
KW - Sleep
UR - http://www.scopus.com/inward/record.url?scp=64449086040&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=64449086040&partnerID=8YFLogxK
U2 - 10.1016/j.sleep.2008.03.004
DO - 10.1016/j.sleep.2008.03.004
M3 - Article
C2 - 18550428
AN - SCOPUS:64449086040
SN - 1389-9457
VL - 10
SP - 374
EP - 377
JO - Sleep Medicine
JF - Sleep Medicine
IS - 3
ER -