摘要肌萎缩侧索硬化(ALS)是一种以上、下运动神经元选择性变性为特征的神经退行性疾病。虽然ALS以运动系统障碍为最突出临床表现,但随着对疾病发病机制认识和临床检测的深入了解,越来越多的ALS患者被发现合并有非运动症状,如感觉障碍等。目前已有电生理、神经病理、神经影像、动物模型模拟、遗传学证据等多种方法的研究结果均提示ALS患者存在感觉神经系统受累。本文对ALS感觉神经系统受累的症状及相关检测方法进行综述,并着重总结与感觉神经系统受累相关的遗传学信息。
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abstractsAmyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the degeneration of upper and lower motor neurons selectively. Although the motor system lesion is the most predominant clinical manifestation of ALS, with the progression of the understanding of the pathogenesis and clinical detection of the disease, more and more patients are found to have extra-motor features of ALS, such as somatosensory involvement, etc. The research results demonstrated that ALS might be a kind of disorder combined with sensory disturbance according to the electrophysiology, neuropathology, neuroimaging, animal model simulation, genetic evidence, and other methods detected. We, herein, review the prevalence and detection methods especially the aspect of genetic associations implicated in the sensory nerve disturbance of ALS.
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