The emerging picture of ALS: a multisystem, not only a "motor neuron disease.

IF 0.8 4区 医学 Q4 NEUROSCIENCES
Vincenzo Silani, Albert Ludolph, Francesco Fornai
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引用次数: 16

Abstract

Amyotrophic lateral sclerosis (ALS) is traditionally considered a disease affecting exclusively motor neurons: compelling evidence points now towards additional involvement of extramotor functions. Beside the cognitive-behavioural disturbances, many ALS patients express extrapyramidal deficits: neuropathological findings fully support the multisystem brain degeneration. The therapeutical option to treat the multisystemic character of ALS represents an additional difficult task in absence of sensitive biomarkers or better understanding of the pathophysiological mechanisms of the disease. Future clinical trials need to identify subgroups of patients, representing the post-hoc analysis after a trial the extreme effort to define sensitive ALS patients to new treatments, as the edaravone story seems to demonstrate.

肌萎缩侧索硬化症的新图景:一个多系统疾病,而不仅仅是一个“运动神经元疾病”。
肌萎缩性侧索硬化症(ALS)传统上被认为是一种仅影响运动神经元的疾病:现在有令人信服的证据表明,运动外功能也参与其中。除了认知行为障碍外,许多ALS患者还表现出锥体外系缺陷:神经病理学结果充分支持多系统脑变性。在缺乏敏感的生物标志物或更好地了解该疾病的病理生理机制的情况下,治疗ALS的多系统特征的治疗选择是一项额外的艰巨任务。未来的临床试验需要确定患者的亚组,这代表了试验后的事后分析,需要付出极大的努力来确定对新治疗敏感的ALS患者,正如依达拉奉的故事所证明的那样。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Archives Italiennes De Biologie
Archives Italiennes De Biologie 医学-神经科学
CiteScore
2.10
自引率
30.00%
发文量
12
审稿时长
>12 weeks
期刊介绍: Archives Italiennes de Biologie - a Journal of Neuroscience- was founded in 1882 and represents one of the oldest neuroscience journals in the world. Archives publishes original contributions in all the fields of neuroscience, including neurophysiology, experimental neuroanatomy and electron microscopy, neurobiology, neurochemistry, molecular biology, genetics, functional brain imaging and behavioral science. Archives Italiennes de Biologie also publishes monographic special issues that collect papers on a specific topic of interest in neuroscience as well as the proceedings of important scientific events. Archives Italiennes de Biologie is published in 4 issues per year and is indexed in the major collections of biomedical journals, including Medline, PubMed, Current Contents, Excerpta Medica.
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