Urbanits S, Budka H
Klinischen Institut für Neurologie, Universität Wien.
Wien Med Wochenschr. 1996;146(9-10):199-200.
We analyze neuronal cytopathology and secondary reactions in spinal-muscular atrophy (SMA) in comparison with amyotrophic lateral sclerosis (ALS). In a series of SMA and ALS cases, immunohistochemistry was performed on spinal cord sections for neuronal, astroglial and microglial antigens, ubiquitin and tau proteins. Swollen motoneurons staining for phosphorylated neurofilament proteins are seen in most SMA but few ALS cases. Ubiquitinated inclusions are found only in ALS. In SMA, glial bundles are prominent in anterior roots, to lesser extent in posterior roots. In ALS, glial bundles are seen only in some cases. While basic histopathologies are similar in both types of motor neurone diseases, neuronal cytoskeletal pathology is more prominent in SMA, possibly reflecting a more acute degenerative process. The presence of axon spheroids and glial bundles also in posterior horns/roots of both types of motor neurone disease suggests spread of degenerative pathology beyond the motor system.
我们将脊髓性肌萎缩症（SMA）与肌萎缩侧索硬化症（ALS）相比较，分析了神经元细胞病理学及继发反应。在一系列SMA和ALS病例中，对脊髓切片进行免疫组织化学检测，以检测神经元、星形胶质细胞和小胶质细胞抗原、泛素和tau蛋白。在大多数SMA病例中可见磷酸化神经丝蛋白染色的肿胀运动神经元，但在少数ALS病例中可见。泛素化包涵体仅在ALS中发现。在SMA中，胶质束在前根中突出，在后根中程度较轻。在ALS中，仅在某些病例中可见胶质束。虽然这两种运动神经元疾病的基本组织病理学相似，但神经元细胞骨架病理学在SMA中更为突出，这可能反映了一个更急性的退行性过程。两种运动神经元疾病的后角/后根中也存在轴突球和胶质束，这表明退行性病理学超出了运动系统的范围。
