Diffuse thalamic degeneration in fatal familial insomnia. A morphometric study

Brain Res. 1997 Oct 10;771(1):154-8. doi: 10.1016/s0006-8993(97)00902-5.

Abstract

A morphometric investigation disclosed most thalamic nuclei severely degenerated in two patients with fatal familial insomnia. Associative and motor nuclei lost 90% neurons, and limbic-paralimbic, intralaminar and reticular nuclei lost 60%. These findings point to the disorganization of most thalamic circuits as a condition necessary for the sleep-wake rhythm being affected.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Asparagine
  • Aspartic Acid
  • Codon
  • Creutzfeldt-Jakob Syndrome / pathology
  • Fatal Outcome
  • Female
  • Humans
  • Male
  • Middle Aged
  • Nerve Degeneration / genetics*
  • Nerve Degeneration / pathology*
  • Neurons / pathology
  • Point Mutation
  • Prions / genetics*
  • Reference Values
  • Sleep Initiation and Maintenance Disorders / genetics*
  • Sleep Initiation and Maintenance Disorders / pathology*
  • Thalamic Nuclei / pathology*

Substances

  • Codon
  • Prions
  • Aspartic Acid
  • Asparagine