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运动神经元病(Motor Neuron Disease,MND)是以损害脊髓前角、脑 干颅神经运动核和锥体束为主的一组 慢性进行性变性疾病。临床以上或 (和)下运动神经元损害引起的瘫痪为 主要表现。目前在分子生物学、免疫、 生化及病毒感染、金属类中毒等病因 方面取得了一定的研究进展,已公认 SOD1基因突变与肌萎缩侧索硬化 (amyotrophic lateral sclerosis,ALS) 有关。新致病基因的发现并用于ALS 研究基因型和表现型动物模型的建立 等,为MND研究提供了良好的平台。 在MND中以ALS最常见,在国内外 研究的也最多,现就其诊治进展综述 如下:
Motor Neuron Disease (MND) is a group of chronic progressive degenerative diseases that mainly damage the anterior horn of the spinal cord, the nucleus of cranial stem cranial nerves, and the pyramidal tract. Clinical manifestations of paralysis caused by motor neuron damage above or below (and). At present, some progress has been made in molecular biology, immunology, biochemistry and virus infection, metal poisoning and other causes. It has been recognized that SOD1 gene mutation is associated with amyotrophic lateral sclerosis (ALS). The discovery of new virulence genes and their application in the development of ALS research genotypes and phenotype animal models provide a good platform for MND research. The most common in the MND to ALS, most of the research at home and abroad, is now on its diagnosis and treatment progress are summarized as follows: