Department of Internal Medicine, Ribeirão Preto School of Medicine, University of São Paulo, São Paulo, Brazil.
Transl Stroke Res. 2012 Sep;3(3):331-40. doi: 10.1007/s12975-012-0177-6. Epub 2012 May 3.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive motor neuron death, leading to muscle atrophy, paralysis, and death usually within 3 to 5 years after diagnosis. Most cases are sporadic, with still undefined etiopathogenesis. Both the innate and adaptive immune systems are involved in ALS, with special participation of T lymphocytes and microglia. Inflammation plays a dual role in the disease, protective and T regulatory cell rich in the early stages and deleterious as disease progresses. Attempts to modulate immune/inflammatory system response are reported in the literature, and while beneficial effects are achieved in ALS animal models, results of most clinical trials have been disappointing. The impaired blood-brain barrier is an important feature in the pathogenesis of ALS and likely affects the immune system response. The present review describes the role of the immune system in ALS pathogenesis and the tight coupling of immunity and central nervous system barrier function.
肌萎缩侧索硬化症（ALS）是一种神经退行性疾病，其特征是运动神经元进行性死亡，导致肌肉萎缩、瘫痪，并在诊断后通常在 3 至 5 年内死亡。大多数病例为散发性，其病因和发病机制仍未明确。固有免疫和适应性免疫系统均参与 ALS 的发病，其中 T 淋巴细胞和小胶质细胞的参与具有特殊性。炎症在疾病中起双重作用，在疾病早期富含保护性和 T 调节细胞，但随着疾病的进展则具有破坏性。文献中报道了针对免疫/炎症系统反应的调节尝试，尽管在 ALS 动物模型中取得了有益效果，但大多数临床试验的结果令人失望。血脑屏障受损是 ALS 发病机制中的一个重要特征，可能会影响免疫系统的反应。本综述描述了免疫系统在 ALS 发病机制中的作用以及免疫与中枢神经系统屏障功能的紧密耦合。
