Fatal familial insomnia and agrypnia excitata

Elio Lugaresi, Federica Provini

Research output: Contribution to journalArticlepeer-review


This review summarizes the pioneering steps culminating in the identification of a novel disease, fatal familial insomnia (FFI), a hereditary prion disease. Together with Morvan's chorea and delirium tremens, FFI is characterized by an inability to sleep associated with motor and autonomic over activation. We named this pattern agrypnia excitata, a syndrome caused by a dysfunction in thalamolimbic circuits. This review highlights the strategic role of the limbic thalamus in the central autonomic network running from the limbic cortex to the lower brainstem and regulating sleep and wakefulness.

Original languageEnglish
Pages (from-to)145-152
Number of pages8
JournalReviews in Neurological Diseases
Issue number3
Publication statusPublished - Jun 2007


  • Agrypnia excitata
  • Delirium tremens
  • Fatal familial insomnia
  • Morvan's chorea
  • Thalamus

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology


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