A case of frontotemporal dementia and parkinsonism of early onset with progressive supranuclear palsy-like features

We report a patient with frontotemporal degeneration and parkinsonism with mental retardation. The patient was a 54-year-old man who had parkinsonism that resembled progressive supranuclear palsy, frontotemporal degeneration and myoclonus. His family included many affected members. Neuropathological ly, there was degeneration of the frontal and temporal cortices, the basal ganglia, the brainstem and the cerebellum. Microscopically, neuronal loss w as severe in the frontal and temporal cortex, the globus pallidus, substant ia nigra, red nucleus and dentate nucleus. Fibrillary changes were found in neurons and glia that were immunostained for tau. Although we could not de fine the genetic abnormalities, we thought that this case might have involv ed frontotemporal dementia and parkinsonism linked to chromosome 17.