Neurofibrillary pathology in progressive supranuclear palsy (PSP).

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.