Agrypnia excitata: clinical features and pathophysiological implications.
Polysomnographic study of sleeplessness and oneiricisms in the alcohol withdrawal syndrome. Plazzi G, Montagna P, Meletti S, Lugaresi E. Morvan’s syndrome: peripheral and central nervous system and cardiac involvement with antibodies to voltage-gated potassium channels. Liguori R, Vincent A, Clover L, Avoni P, Plazzi G, Cortelli P, et al.
#Fatal familial insomnia serial
Pre-symptomatic diagnosis in fatal familial insomnia: serial neurophysiological and 18fdg-pet studies. 673–775.Ĭortelli P, Perani D, Montagna P, Gallassi R, Tinuper P, Provini F, et al. New York: Cold Spring Harbor Laboratory Press 2004. Kong Q, Surewicz WK, Petersen RB, Zou W, Chen SG, Gambetti P, et al. Fatal familial insomnia and dysautonomia with selective degeneration of thalamic nuclei. Lugaresi E, Medori R, Montagna P, Baruzzi A, Cortelli P, Lugaresi A, et al. These three conditions (FFI, MS and DT) present different pathophysiological mechanisms but share the same thalamolimbic impairment of which AE is one of the possible clinical presentations.įFI, and consequently AE, is a model for the investigation of the essential role of the thalamus in the organization of body homeostasis, integrating both sleep and autonomic function control. Sleep loss, autonomic, and motor hyperactivation define agrypnia excitata (AE), which is not exclusive to FFI, but described also in Morvan syndrome (MS) and delirium tremens (DT). Cardiovascular and thermoregulatory systems are the most frequently affected, confirming the increased sympathetic drive with preserved parasympathetic responses. FFI is characterized by physiological sleep loss, polygraphically appearing as a slow wave sleep loss, autonomic and motor hyperactivation with peculiar episodes of oneiric stupor.ĭysfunction of the autonomic system is a great burden for FFI patients consisting of sympathetic overactivation, dysregulation of its physiological responses, and disruption of circadian rhythms. Fatal familial insomnia (FFI) is a hereditary prion disease linked to a missense mutation at codon 178 of the prion-protein gene ( PRNP) located on chromosome 20, determining severe and selective atrophy of mediodorsal and anteroventral thalamic nuclei.
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