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运动神经元疾病(MND)是一种多基因疾病。最常见的变异型为非遗传性肌萎缩侧索硬化(ALS),其病因或治疗方法尚未明确。最近报道家族型ALS由于铜/锌超氧化物歧化酶而发生基因突变,提示自由基对运动神经元的毒性;有些类型的MND可能由免疫介导起病。特别是高水平的抗神经节苷脂抗体(抗GM_1抗体)可能是可治型MND的标志,已引起注意;一些有传导障碍(Conduction block,CB)的MND也有治疗反应,这组综合征被称为多发性运动神经元病(multifocal motor neuropathy,MMN)。
Motor neuron disease (MND) is a polygenic disease. The most common variant is non-heritable amyotrophic lateral sclerosis (ALS), the etiology or treatment is not yet clear. Recent reports of familial ALS mutations due to copper / zinc superoxide dismutase, suggesting that free radicals on motor neurons toxicity; some types of MND may be mediated by immune-mediated disease. In particular, high levels of anti-ganglioside antibodies (anti-GMl antibodies) may be hallmarks of a modifiable MND that has attracted attention; some MND with Conduction block (CB) also have therapeutic responses that are Known as multiple motor neuron disease (multifocal motor neuropathy, MMN).