Bellosta-Diago E, Viloria-Alebesque A, Santos-Lasaosa S, Lopez Del Val L J
Hospital Clinico Universitario Lozano Blesa, 50009 Zaragoza, Espana.
Rev Neurol. 2017 Nov 1;65(9):415-422.
Disorders affecting sleep and the circadian rhythm, autonomic clinical signs and symptoms, and neuroendocrine alterations are frequent characteristics in Huntington's disease, some of which present in early stages of the disease. It is reasonable to think that some of these features could result from a hypothalamic dysfunction affecting the centre regulating sleep, metabolism and the autonomic nervous system.
The study presents the evidence available to date that suggests the involvement of a hypothalamic disorder in Huntington's disease.
Histopathological, hormonal and neuroimaging research relates this area of the brain to Huntington's disease. The experimental findings and those obtained with animal models or in studies conducted with patients are summarised. Likewise, the clinical repercussions (sleep and circadian rhythm disorders, psychiatric and cognitive pathologies, and the clinical signs and symptoms linked to autonomic dysfunction) secondary to possible involvement of the hypothalamus in this disease are also described.
The hypothalamus acts as a centre that integrates the neuroendocrine and autonomic functions, and plays a significant role in cognitive and behavioural signs and symptoms. Disorders of this type have been highlighted in Huntington's disease. Further studies are needed to elucidate the role and scope of this region of the brain in this disease.
影响睡眠和昼夜节律、自主神经临床体征和症状以及神经内分泌改变是亨廷顿舞蹈病的常见特征，其中一些在疾病早期就会出现。有理由认为，这些特征中的一些可能是由于下丘脑功能障碍影响了调节睡眠、代谢和自主神经系统的中枢所致。
本研究展示了迄今为止表明下丘脑功能障碍与亨廷顿舞蹈病有关的现有证据。
组织病理学、激素和神经影像学研究将大脑的这一区域与亨廷顿舞蹈病联系起来。总结了实验结果以及在动物模型或患者研究中获得的结果。同样，还描述了下丘脑可能参与该疾病继发的临床影响（睡眠和昼夜节律障碍、精神和认知病理学以及与自主神经功能障碍相关的临床体征和症状）。
下丘脑作为整合神经内分泌和自主神经功能的中枢，在认知和行为体征及症状中发挥重要作用。这种类型的功能障碍在亨廷顿舞蹈病中已得到凸显。需要进一步研究以阐明大脑这一区域在该疾病中的作用和范围。
