Pallidonigroluysian atrophy: Clinicopathological and immunohistochemical studies

Three autopsied cases of pallidonigroluysian atrophy (PNLA) were studied clinicopathologically and 11 previously reported cases were reviewed for the purpose of clarifying the clinicopathological characteristics of the disease. The main clinical symptoms are parkinsonism, predominantly akinesia or pure akinesia with or without effective levodopa therapy. Major lesions approximately were confined to the globus pallidus, subthalamic nucleus and substantia nigra. Gallyas-Braak method and immunohistochemistry with a monoclonal antibody (mAb) to tau-2 protein (tau-2) revealed a small number of neurofibrillary tangles (NFT) and argyrophilic and tau-2 positive structures within the cytoplasms of the glial cells in the major lesions. Immunohistochemistry with a mAb to Alzheimer NFT (Ab39) demonstrated only a few classical tangles in the globus pallidus in one case. These findings suggest that PNLA can be pathologically differentiated from progressive supranuclear palsy.