Pathologic findings are presented in a case of neurodegenerative disease with a 5-year course of progressive presenile dementia and parkinsonism. At autopsy, atrophy of the cerebral cortex, caudate nucleus, and substantia nigra were observed. Homogeneous eosinophilic intracytoplasmic neuronal inclusions were found, predominantly in the brainstem and cerebellar nuclei. These inclusions were nonargyrophilic and did not stain with antibodies against tau and ubiquitin.