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Diffuse thalamic degeneration in fatal familial insomnia. A morphometric study
Authors:Giorgio Macchi  Giacomina Rossi  Anna Laura Abbamondi  Giorgio Giaccone  Domenico Mancia  Fabrizio Tagliavini  Orso Bugiani
Affiliation:aIstituto Neurologico Carlo Besta, Via Celoria 11, 20133 Milan, Italy;bUniversità Cattolica del Sacro Cuore, Largo Gemelli 8, 00168 Rome, Italy;cClinica Neurologica dell'Università, Viale Bracci 2, 53100 Siena, Italy;dClinica Neurologica dell'Università, Via del Quartiere 4, 43100 Parma, Italy
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.
Keywords:Thalamus   Insomnia   Prion disease
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