Neurodegeneration in the limbic and paralimbic system in progressive supranuclear palsy

Progressive supranuclear palsy is neuropathologically characterized by neuronal degeneration of the basal ganglia, brain stem, and cerebellum. In addition, cortical neuronal degeneration associated with neurofibrillary tangles formation has been identified over wide areas of the brain in patients with progressive supranuclear palsy. We studied the distribution of alpha B-crystallin-positive degenerating neurons in cases with progressive supranuclear palsy, and compared them with those in Pick's disease. Alzheimer's disease, senile dementia of Alzheimer type, and normal aged individuals. A large number of alpha B-crystallin-positive neurons was found in the cerebral cortices of four out of nine patients with progressive supranuclear palsy. In particular, alpha B-crystallin-positive ballooned neurons were frequently observed in deep cortical pyramidal cell layers of the limbic and paralimbic systems in these diseases. The involvement of the limbic and paralimbic systems may thus contribute to personality changes as well as to memory and cognitive impairment in some patients with progressive supranuclear palsy.