The University of Queensland, UQ Centre for Clinical Research, Australia.
Curr Mol Med. 2011 Apr;11(3):246-54. doi: 10.2174/156652411795243450.
Amyotrophic lateral sclerosis (ALS) is a severe progressive neurodegenerative disease. The cause is unknown, but genetic abnormalities have been identified in subjects with familial ALS and also in subjects with sporadic ALS. Environmental factors such as occupational exposure have been shown to be risk factors for the development of ALS. Patients differ in their clinical features and differ in the clinical course of disease. Immune abnormalities have been found in the central nervous system by pathological studies and also in the blood and CSF of subjects with ALS. Inflammation and immune abnormalities are also found in animals with a model of ALS due to mutations in the SOD1 gene. Previously it has been considered that immune abnormalities might contribute to the pathogenesis of disease. However more recently it has become apparent that an immune response can occur as a response to damage to the nervous system and this can be protective.
肌萎缩侧索硬化症（ALS）是一种严重的进行性神经退行性疾病。其病因不明，但在家族性 ALS 患者和散发性 ALS 患者中已发现遗传异常。环境因素，如职业暴露，已被证明是 ALS 发病的危险因素。患者的临床特征不同，疾病的临床过程也不同。病理研究发现中枢神经系统存在免疫异常，ALS 患者的血液和 CSF 中也存在免疫异常。由于 SOD1 基因突变，ALS 动物模型中也存在炎症和免疫异常。以前认为免疫异常可能有助于疾病的发病机制。然而，最近越来越明显的是，免疫反应可能是对神经系统损伤的一种反应，这种反应具有保护作用。
