Familial and sporadic fatal insomnia

@article{Montagna2003FamilialAS,
  title={Familial and sporadic fatal insomnia},
  author={P. Montagna and P. Gambetti and P. Cortelli and E. Lugaresi},
  journal={The Lancet Neurology},
  year={2003},
  volume={2},
  pages={167-176}
}
  • P. Montagna, P. Gambetti, +1 author E. Lugaresi
  • Published 2003
  • Psychology, Medicine
  • The Lancet Neurology
  • Familial fatal insomnia (FFI)--a hereditary prion disease caused by a mutation at codon 178 of the prion-protein (PrP) gene (PRNP) that leads to a D178N substitution in the protein--and its sporadic form, sporadic fatal insomnia (SFI), have similar disease phenotypes. Both disorders have clinical features of disrupted sleep (loss of sleep spindles and slow-wave sleep and enacted dreams during rapid-eye-movement sleep), autonomic hyperactivation, and motor abnormalities (myoclonus, ataxia… CONTINUE READING
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