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ALS患者下丘脑萎缩与疾病定义症状的发病相关

Hypothalamic atrophy correlates with onset of disease-defining symptoms in patients with ALS
课程网址: http://videolectures.net/encals2017_gorges_hypothalamic_atrophy/  
主讲教师: Martin Gorges
开课单位: 德国乌尔姆大学
开课时间: 2017-07-21
课程语种: 英语
中文简介:
背景：能量代谢的改变已在ALS的背景下被描述，但缺乏下丘脑参与的直接证据。下丘脑在控制能量代谢中起着关键作用。目的：研究显性ALS和无症状ALS突变携带者下丘脑形态的改变是否和病程有关。方法：在一个大型单中心队列中获得基于高分辨率全脑T1加权3D-MRI数据以及临床和实验室参数，包括270例ALS患者、32例无症状ALS突变携带者和116例健康对照。根据一个完善的程序，对下丘脑体积进行半手动定量，该程序经过优化，可灵敏地检测下丘脑体积的变化。所有受试者的下丘脑体积均由经验丰富的评分员测量，该评分员对任何临床信息均不知情。确定的下丘脑体积根据颅内体积进行标准化，并根据年龄进行校正。结果：与对照组相比，显性ALS患者（p<0.0001）和无症状ALS突变携带者（p<0.01）的下丘脑体积均显著萎缩。与全脑萎缩、代谢指数（包括BMI）或下丘脑相关激素（瘦素、脂联素）无显著相关性。相反，疾病的发病与下丘脑体积显著相关（p=0.012），下丘脑萎缩越少，存活率越高（p=0.022）。结论：即使在ALS的无症状期，下丘脑也有选择性萎缩。下丘脑体积的减少很可能与ALS症状的发生有关，并且可能是生存的一个预后因素
课程简介: Background: Alterations in energy metabolism have been described in the context of ALS but direct evidence of hypothalamic involvement is lacking. The hypothalamus plays a key role in controlling the energy metabolism. Objective: To study whether possible morphologic alterations of the hypothalamus in manifest ALS and asymptomatic ALS mutation carriers are associated with the course of the disease. Methods: High-resolution whole-brain based T1-weighted 3D-MRI data together with clinical and laboratory parameters were acquired in a large monocentric cohort including 270 manifest ALS patients, 32 asymptomatic ALS mutation carriers, and 116 healthy controls. The hypothalamic volumes were semimanually quantified following a well-established procedure that was optimized to sensitively detect hypothalamic volume alterations. The hypothalamic volumes for all subjects were measured by an experienced rater who was blinded for any clinical information. The determined hypothalamic volumes were normalized for the intracranial volume and corrected for age. Results: The hypothalamic volumes were significantly atrophied in both manifest ALS patients (p<0.0001) and asymptomatic ALS mutation carriers (p<0.01) compared with controls. There was no significant correlation with global brain atrophy, metabolic indices (among others BMI), or hypothalamic-associated hormones (leptin, adiponectin). In contrast, the disease onset was significantly correlated with the hypothalamic volume (p=0.012) and survival was better (p=0.022) the less the hypothalamus was atrophied. Conclusions: The hypothalamus appears to be selectively atrophied even in an asymptomatic phase of ALS. The volume loss of the hypothalamus is likely to be associated with the onset of ALS-defining symptoms and possibly a prognostic factor for survival
关 键 词: 下丘脑; ALS; 下丘脑萎缩
课程来源: 视频讲座网
数据采集: 2021-12-25：zkj
最后编审: 2021-12-25：zkj
阅读次数: 67

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