Diffuse thalamic degeneration in fatal familial insomnia. A morphometric study.
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.
Macchi
G
G
Istituto Neurologico Carlo Besta, Milan, Italy.
Rossi
G
G
Abbamondi
A L
AL
Giaccone
G
G
Mancia
D
D
Tagliavini
F
F
Bugiani
O
O
D000328:Adult; D001216:Asparagine; D001224:Aspartic Acid; D003062:Codon; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009410:Nerve Degeneration / Q000235:genetics* / Q000473:pathology*; D009474:Neurons / Q000473:pathology; D017354:Point Mutation; D011328:Prions / Q000235:genetics*; D012016:Reference Values; D007319:Sleep Initiation and Maintenance Disorders / Q000235:genetics* / Q000473:pathology*; D013787:Thalamic Nuclei / Q000473:pathology*
D003062:Codon; D011328:Prions; D001224:Aspartic Acid; D001216:Asparagine