Cortelli P, Parchi P, Contin M, Pierangeli G, Avoni P, Tinuper P, Montagna P, Baruzzi A, Gambetti P L, Lugaresi E
Neurological Institute, University of Bologna Medical School, Italy.
Clin Auton Res. 1991 Mar;1(1):15-21. doi: 10.1007/BF01826053.
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.
对两名患有致命性家族性失眠症的患者的心血管系统自主控制情况进行了评估。患者1经尸检确诊。在静息状态下，患者1的血压和心率高于对照组；两名患者的血浆去甲肾上腺素水平均升高。心血管反射评估表明压力感受器反射通路完整，但对某些刺激（体位改变、瓦尔萨尔瓦动作、等长握力）的血压和生化反应过度。静脉注射去甲肾上腺素无升压反应，对阿托品反应增强，对可乐定的降压和镇静作用减弱。总体而言，这些结果表明自主控制失衡，副交感神经功能保留，背景交感神经活动较高且受刺激时交感神经活动增强。这些生理、生化和药理学数据，以及该疾病已知的神经病理学发现，强调了丘脑在调节人类心血管功能自主控制中可能发挥的作用。
