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介绍运动神经元病(motor neuron disease,MND)的诊断方法、常用诊断标准和分类.肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是MND中最为常见的形式.脑干和脊髓各节段的上、下运动神经元受累体征是诊断MND/ALS的重要依据,而肌电图是判断亚临床下运动神经元受累的重要辅助检查.国际公认的ALS诊断标准共有3种,依次为El Escorial诊断标准、Airlie House诊断标准(又称修订版El Escorial诊断标准)和Awaji-shima电生理诊断标准.MND存在多种分类方法,根据病因可将其分为家族性和散发性;根据起病部位及临床表现可分为肢体起病型ALS、球部起病型ALS、进行性肌萎缩、原发侧索硬化、连枷臂综合征和连枷腿综合征等多种类型.

This presentation introduced the diagnostic approach, common diagnostic criteria and classification of motor neuron disease (MND). Amyotrophic lateral sclerosis (ALS) is the most common type in MND. Involvement of upper and lower motor neurons in the brain stem and spinal cord is the most important signs for the diagnosis of MND/ALS, and electromyography is an important examination to determine subclinical lower motor neuron involvement. There are three internationally recognized diagnostic criteria, the El Escorial criteria, the Airlie House criteria (revised El Escorial criteria) and the Awaji-shima electrodiagnostic criteria. There are several methods for classification for MND. MND can be classified as familial and sporadic MND based on the cause of disease, or can be classified as limb-onset ALS, bulbar-onset ALS, progressive muscular atrophy, primary lateral sclerosis, flail arm syndrome, flail leg syndrome and other types by the site of onset and clinical features.