Dupuis Luc, Petersen Åsa, Weydt Patrick
Faculty of Medicine, University of Strasbourg, Strasbourg, France.
Translational Neuroendocrine Research Unit, Department of Experimental Medical Sciences, Lund University, Lund, Sweden.
Handb Clin Neurol. 2018;157:749-760. doi: 10.1016/B978-0-444-64074-1.00046-X.
Amyotrophic lateral sclerosis (ALS) is the major adult-onset motor neuron disease, and is clinically, pathologically, and genetically associated with frontotemporal dementia, the second cause of dementia in the elderly. Here, we review the evidence linking thermoregulation and ALS. Indeed, while ALS is not classically associated with defective thermoregulatory function, its progression severely affects key brain regions controlling body temperature and impacts multiple sensors and effectors of this homeostatic function. Furthermore, animal models of ALS display disturbed thermoregulation as a consequence of disrupted energy homeostasis. All these lines of indirect evidence call for studies directly addressing the body temperature regulatory system, both as a potential biomarker and as a possible modifier of disease progression in ALS.
肌萎缩侧索硬化症（ALS）是主要的成人发病的运动神经元疾病，在临床、病理和基因方面与额颞叶痴呆相关，后者是老年人痴呆的第二大病因。在此，我们综述了将体温调节与ALS联系起来的证据。实际上，虽然ALS通常与体温调节功能缺陷无关，但其进展会严重影响控制体温的关键脑区，并影响这种稳态功能的多个传感器和效应器。此外，ALS动物模型由于能量稳态破坏而出现体温调节紊乱。所有这些间接证据都呼吁开展直接针对体温调节系统的研究，将其作为一种潜在的生物标志物以及ALS疾病进展的可能调节因素。
