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

Federica Provini, Roberto Vetrugno, Giulia Pierangeli, Pietro Cortelli, Giovanni Rizzo, Alessandro Filla, Caterina Strisciuglio, Roberto Gallassi, Pasquale Montagna

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)374-377
Number of pages4
JournalSleep Medicine
Issue number3
Publication statusPublished - Mar 2009


  • Circadian rhythm
  • Gerstmann-Sträussler-Scheinker
  • Neurodegerneative diseases
  • Polysomnography
  • Prion diseases
  • Sleep

ASJC Scopus subject areas

  • Medicine(all)


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