Lugaresi E, Tobler I, Gambetti P, Montagna P
Institute of Clinical Neurology, University of Bologna, Italy.
Brain Pathol. 1998 Jul;8(3):521-6. doi: 10.1111/j.1750-3639.1998.tb00173.x.
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.
致死性家族性失眠症（FFI）的关键临床特征，即警觉性降低和注意力缺陷、无法产生脑电图睡眠模式、交感神经功能亢进以及自主神经和激素昼夜节律振荡减弱，与丘脑前腹侧和内侧背侧核的选择性萎缩有关。这些核构成丘脑的边缘部分，连接皮质的边缘和边缘旁区域以及边缘系统中的其他皮质下结构，包括下丘脑。从皮质-边缘控制中释放出来的下丘脑转而以激活功能为主，而非失活功能，包括睡眠丧失、交感神经功能亢进以及随之而来的自主神经昼夜节律和内分泌振荡减弱。这些发现证明，边缘丘脑在从边缘皮质区域延伸至脑干下部的中枢自主神经网络中具有重要地位，该网络以整合的方式调节身体的内环境稳定。
