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肌萎缩侧索硬化症中潜在的皮肤受累:重新审视夏科的观察——肌萎缩侧索硬化症皮肤异常的综述

Potential skin involvement in ALS: revisiting Charcot's observation - a review of skin abnormalities in ALS.

作者信息

Paré Bastien, Gros-Louis François

机构信息

.

出版信息

Rev Neurosci. 2017 Jul 26;28(5):551-572. doi: 10.1515/revneuro-2017-0004.

Abstract

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting motor neurons of the brain and spinal cord, leading to progressive paralysis and death. Interestingly, many skin changes have been reported in ALS patients, but never as yet fully explained. These observations could be due to the common embryonic origin of the skin and neural tissue known as the ectodermal germ layer. Following the first observation in ALS patients' skin by Dr Charcot in the 19th century, in the absence of bedsores unlike other bedridden patients, other morphological and molecular changes have been observed. Thus, the skin could be of interest in the study of ALS and other neurodegenerative diseases. This review summarizes skin changes reported in the literature over the years and discusses about a novel in vitro ALS tissue-engineered skin model, derived from patients, for the study of ALS.

摘要

肌萎缩侧索硬化症(ALS)是一种影响大脑和脊髓运动神经元的神经退行性疾病,会导致进行性瘫痪和死亡。有趣的是,已有许多关于ALS患者皮肤变化的报道,但至今尚未得到充分解释。这些观察结果可能归因于皮肤和神经组织共同的胚胎起源,即外胚层胚层。自19世纪夏科医生首次观察到ALS患者的皮肤以来,发现与其他卧床患者不同,ALS患者不存在褥疮,之后还观察到了其他形态学和分子变化。因此,皮肤可能在ALS和其他神经退行性疾病的研究中具有重要意义。这篇综述总结了多年来文献中报道的皮肤变化,并讨论了一种源自患者的新型体外ALS组织工程皮肤模型,用于ALS的研究。

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