Cervós-Navarro J, Schumacher K
Institute of Neuropathology, Free University of Berlin, Federal Republic of Germany.
J Neural Transm Suppl. 1994;42:153-64. doi: 10.1007/978-3-7091-6641-3_12.
In progressive supranuclear palsy (PSP), globose neurofibrillary tangles (NFT) are found in the subcortical areas and occasionally in the central cortex and spinal cord. An inverse relationship was found between the degree of neuronal loss and the presence of NFT. It has been postulated that NFT comes first and atrophy as a secondary event. Others authors have reported that the neurologic findings are associated with the presence of carcinomas and the CNS changes can be assessed as a paraneoplastic effect. In PSP the neuritic changes are mainly located in the basal ganglia and composed of straight filaments and tubules, different from the paired helical filaments found in the Alzheimer's disease, suggesting that they are formed of a new type of fibrous protein. In addition immunohistochemistry preparations using antibodies against tau and ubiquitin reveal an antigenic profile similar to early NFT in dementia of Alzheimer's type. These findings support the hypothesis that these changes may reflect different types of non-specific cytoskeletal disorganization.
在进行性核上性麻痹（PSP）中，球状神经原纤维缠结（NFT）见于皮质下区域，偶尔也见于中央皮质和脊髓。研究发现神经元丢失程度与NFT的存在呈负相关。据推测，NFT先出现，萎缩是继发事件。其他作者报告称，神经系统表现与癌的存在有关，中枢神经系统变化可被评估为副肿瘤效应。在PSP中，神经炎性改变主要位于基底神经节，由直丝和微管组成，不同于阿尔茨海默病中发现的双螺旋丝，这表明它们由一种新型纤维蛋白构成。此外，使用抗tau和泛素抗体的免疫组织化学制剂显示出与阿尔茨海默病型痴呆早期NFT相似的抗原谱。这些发现支持了这样一种假说，即这些变化可能反映了不同类型的非特异性细胞骨架紊乱。
