An atypical case of sporadic fatal insomnia

L. Priano, G. Giaccone, M. Mangieri, G. Albani, L. Limido, A. Brioschi, L. Pradotto, L. Orsi, P. Mortara, P. Fociani, A. Mauro, F. Tagliavini

Research output: Contribution to journalArticle

Abstract

Fatal insomnia is a rare human prion disease characterised by sleep-wake disturbances, thalamic degeneration and deposition of type 2 disease-specific prion protein (PrPSc). This report details a patient with sporadic fatal insomnia who exhibited cerebral deposition of type 1 PrPSc and neuropathological changes largely in the basal ganglia. Previous damage of this brain region by a surgically removed colloid cyst and the insertion of two intracerebral shunts may have influenced the distribution of PrPSc through a chronic inflammatory process. These findings add to our knowledge of the phenotypic variability of human prion diseases with prominent sleep disturbances.

Original languageEnglish
Pages (from-to)924-927
Number of pages4
JournalJournal of Neurology, Neurosurgery and Psychiatry
Volume80
Issue number8
DOIs
Publication statusPublished - Aug 2009

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ASJC Scopus subject areas

  • Clinical Neurology
  • Psychiatry and Mental health
  • Surgery
  • Medicine(all)

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