Yoshino H
Department of Neurology, Kohnodai Hospital.
Nihon Rinsho. 1994 Nov;52(11):2984-9.
Amyotrophic lateral sclerosis (ALS) is a devastating human neurologic disorder which causes atrophy and weakness of skeletal muscles leading to death. Although pathogenesis of most cases of ALS is not known yet, large number of evidence suggest autoimmune mechanisms through clinical, pathological, and laboratory findings, for example, high frequency of the presence of serum monoclonal gammopathy, association of thyroid diseases, and lymphocytic infiltration of brain and spinal cord tissues of patients. Especially recent findings of IgG antibodies against calcium channels and IgM anti-GM1 antibodies are important to elucidate the pathogenesis of ALS. Although conventional immunotherapy may not be effective, multifocal motor neuropathy (MMN) which simulates ALS should be carefully examined because MMN is a treatable disorder.
肌萎缩侧索硬化症(ALS)是一种毁灭性的人类神经系统疾病,会导致骨骼肌萎缩和无力,最终导致死亡。尽管大多数ALS病例的发病机制尚不清楚,但大量证据通过临床、病理和实验室检查结果表明存在自身免疫机制,例如血清单克隆丙种球蛋白病的高发病率、甲状腺疾病的关联以及患者脑和脊髓组织的淋巴细胞浸润。特别是最近发现的针对钙通道的IgG抗体和抗GM1 IgM抗体对于阐明ALS的发病机制很重要。尽管传统免疫疗法可能无效,但应仔细检查疑似ALS的多灶性运动神经病(MMN),因为MMN是一种可治疗的疾病。