NR ACWU
AU Cortelli,P.; Parchi,P.; Contin,M.; Pierangeli,G.; Avoni,P.; Tinuper,P.; Montagna,P.; Baruzzi,A.; Gambetti,P.L.; Lugaresi,E.
TI Cardiovascular dysautonomia in fatal familial insomnia
QU Clinical Autonomic Research 1991 Mar; 1(1): 15-21
PT journal article
AB Autonomic control of the cardiovascular system was assessed in two patients with Fatal Familial Insomnia. The diagnosis was confirmed at autopsy in patient 1. In the resting state blood pressure and heart rate were higher than controls in patient 1; plasma noradrenaline levels were elevated in both patients. Evaluation of cardiovascular reflexes indicated intact baroreflex pathways but with exaggerated blood pressure and biochemical responses to certain stimuli (postural change, Valsalva manoeuvre, isometric handgrip). There was no pressor response to intravenously infused noradrenaline, an increased response to atropine and diminished depressor and sedative effects to clonidine. Overall these results are indicative of an unbalanced autonomic control with preserved parasympathetic and higher background and stimulated sympathetic activity. These physiological, biochemical and pharmacological data, together with known neuro-pathological findings in this disorder, emphasize the possible role played by the thalamus in regulating autonomic control of cardiovascular function in man.
MH Adult; Autonomic Nervous System/physiopathology; Blood Pressure; Cardiovascular System/*physiopathology; Case Report; Clonidine/therapeutic use; Dysautonomia, Familial/drug therapy/*physiopathology; Epinephrine/blood; Female; Heart Rate; *Hemodynamics; Human; Middle Age; Norepinephrine/blood; Reference Values; Respiration; Sleep Initiation and Maintenance Disorders/complications/*physiopathology; Valsalva's Maneuver
AD
Pietro Cortelli, Piero Parchi, Manuela Contin, Giulia Pierangeli, Patrizia Avoni, Paolo Tinuper, Pasquale Montagna, Agostino Baruzzi, Pier Luigi Gambetti, Neurological Institute of the University of Bologna
Medical School, Bologna, Italy; Elio Lugaresi, Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, USA
SP englisch
PO England