Hansen P R, Werdelin L M
Statens Seruminstitut, Autoimmunlaboratoriet, København.
Ugeskr Laeger. 1991 Feb 18;153(8):565-6.
Amyotrophic lateral sclerosis is characterized by degeneration of the motor neurones in the central nervous system and, as a rule, the condition results in rapid incapacity and death. The etiology is unknown but experimental results from recent years suggest that immunological mechanisms are of pathophysiological significance. Gangliosides constitute an important membrane component in nerve tissue and the majority of patients probably have high titres of circulation polyclonal IgM-antibodies to these compounds, particularly gangliosides GM1 and GD1a. The antibody titre appears to be correlated with the clinical condition and selective immune suppression (e.g. with cyclophosphamide) may possibly be of therapeutic value.
肌萎缩侧索硬化症的特征是中枢神经系统中的运动神经元退化，通常情况下，这种疾病会导致迅速丧失能力和死亡。病因不明，但近年来的实验结果表明免疫机制具有病理生理学意义。神经节苷脂是神经组织中的一种重要膜成分，大多数患者可能对这些化合物，尤其是神经节苷脂GM1和GD1a，有高滴度的循环多克隆IgM抗体。抗体滴度似乎与临床状况相关，选择性免疫抑制（如使用环磷酰胺）可能具有治疗价值。
