Engelhardt J I, Appel S H
Department of Neurology, Baylor College of Medicine, Houston, Tex. 77030.
Arch Neurol. 1990 Nov;47(11):1210-6. doi: 10.1001/archneur.1990.00530110068019.
The spinal cord and motor cortex of patients with amyotrophic lateral sclerosis (ALS) were examined with immunohistochemical methods for the presence of IgG. In 13 of 15 spinal cords, a population of motoneurons stained positively for IgG in a granular pattern, characteristic of binding to the rough endoplasmic reticulum. In 6 of 11 motor cortices, a proportion of pyramidal cells also stained positively for IgG. No such reactivity was noted in motoneurons of control human tissues, although positive IgG staining was present in astrocytes of ALS and control specimens. Reactive microglia and/or macrophages were detected in the territory of degenerating pyramidal tracts and ventral horns. The surface of most of these cells stained positively for IgG, and 50% stained positively for HLA-DR. The accumulation of IgG in motoneurons and the presence of immunologically active macrophages provide additional evidence for the participation of immunologic factors in the pathogenesis of ALS.
采用免疫组织化学方法检查肌萎缩侧索硬化症(ALS)患者的脊髓和运动皮层,以检测IgG的存在。在15个脊髓中的13个中,一群运动神经元以颗粒状模式对IgG呈阳性染色,这是与粗面内质网结合的特征。在11个运动皮层中的6个中,一部分锥体细胞对IgG也呈阳性染色。在对照人体组织的运动神经元中未观察到这种反应性,尽管在ALS和对照标本的星形胶质细胞中存在阳性IgG染色。在退化的锥体束和腹角区域检测到反应性小胶质细胞和/或巨噬细胞。这些细胞中的大多数表面对IgG呈阳性染色,50%对HLA-DR呈阳性染色。运动神经元中IgG的积累以及免疫活性巨噬细胞的存在为免疫因素参与ALS发病机制提供了额外证据。