NR AHOU

AU Lugaresi,E.; Tobler,I.; Gambetti,P.; Montagna,P.

TI The pathophysiology of fatal familial insomnia

QU Brain Pathology 1998 Jul; 8(3): 521-6

PT journal article; review; review, tutorial

AB The key clinical aspects of FFI, i.e. hypovigilance and attention deficit, inability to generate EEG sleep patterns, sympathetic hyperactivity and attenuation of vegetative and hormonal circadian oscillations, are related to selective atrophy of the anteroventral and mediodorsal thalamic nuclei. These nuclei constitute the limbic part of the thalamus interconnecting limbic and paralimbic regions of the cortex and other subcortical structures in the limbic system including the hypothalamus. The hypothalamus released from cortico-limbic control is shifted to a prevalence of activating, as opposed to deactivating, functions including loss of sleep, sympathetic hyperactivity and the attendant attenuation of autonomic circadian and endocrine oscillations. These findings document that the limbic thalamus has a strategic position in the central autonomic network running from the limbic cortical regions to the lower brain stem which regulates the body's homeostasis in an integrated fashion.

ZR 45

MH Autonomic Nervous System Diseases/physiopathology; Electroencephalography; Endocrine Diseases/physiopathology; Human; Prion Diseases/pathology/*physiopathology; Sleep Disorders/physiopathology; Support, Non-U.S. Gov't; Thalamus/pathology/*physiopathology

AD Institute of Clinical Neurology, University of Bologna, Italy. lugaresi@bo.nettuno.it

SP englisch

PO Schweiz

EA pdf-Datei

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