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[肌萎缩侧索硬化症]

[Amyotrophic lateral sclerosis].

作者信息

Moriwaka F

机构信息

Department of Neurology, Hokkaido University School of Medicine, Sapporo, Japan.

出版信息

Hokkaido Igaku Zasshi. 1997 Jan;72(1):21-5.

PMID:9086359
Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive and fatal illness characterized by upper and lower motor neuron degeneration of adults. Recently, many advances are being made in our understanding of the pathogenesis of ALS, which are the role of autoimmunity, the glutamate excitotoxicity, the neurotrophic factor and the defined mutation of superoxide dismutase in familial ALS cases. The therapeutic trials have been carried out more commonly based on the putative pathogenesis described. Among them, riluzole, antiglutamate agent, is proved to have the survival advantage in a controlled double-blind randomized study. These current putative pathogenesis and therapeutic trials of ALS are discussed.

摘要

肌萎缩侧索硬化症(ALS)是一种进行性致命疾病,其特征为成人上下运动神经元变性。最近,我们对ALS发病机制的理解取得了许多进展,包括自身免疫的作用、谷氨酸兴奋性毒性、神经营养因子以及家族性ALS病例中超氧化物歧化酶的特定突变。治疗试验更多地是基于所描述的假定发病机制进行的。其中,利鲁唑(一种抗谷氨酸药物)在一项对照双盲随机研究中被证明具有生存优势。本文将讨论ALS目前的这些假定发病机制和治疗试验。

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