[Familial fatal insomnia: a human prion disease which opens the door to a greater understanding of the thalamus].
In 1986, Lugaresi [1] described fatal familial insomnia (FFI), an inherited prion disease, characterised by untreatable insomnia and dysautonomia. The most severe neuropathological changes have been found in the mediodorsal (MD) and anterior (A) thalamic nuclei. The data lead to think that the thalamus could play an important role in the wake-sleep cycle and other vegetative and endocrine circadian activities, specially MD and A.
Departamento de Morfología, Facultad de Medicina, Universidad Autónoma de Madrid.
F
F
Barriga
J A
JA
Ruiz-Domínguez
J L
JL
Velayos
D000328:Adult; D000818:Animals; D002415:Cats; D002940:Circadian Rhythm / Q000502:physiology*; D003062:Codon / Q000235:genetics; D005260:Female; D005799:Genes, Dominant; D005911:Gliosis / Q000209:etiology / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009474:Neurons / Q000473:pathology; D018621:PrPC Proteins / Q000235:genetics; D017096:Prion Diseases / Q000150:complications / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D012890:Sleep / Q000502:physiology*; D007319:Sleep Initiation and Maintenance Disorders / Q000209:etiology / Q000235:genetics / Q000473:pathology / Q000503:physiopathology*; D013787:Thalamic Nuclei / Q000473:pathology / Q000503:physiopathology*
D003062:Codon; D018621:PrPC Proteins
Insomnio familiar fatal: una enfermedad priónica humana que abre las puertas a un mayor conocimiento del tálamo.