SPORADIC OLIVOPONTOCEREBELLAR ATROPHY WITH SUBCORTICAL DEMENTIA AND HALLUCINATORY PARANOID STATE - REPORT OF AN AUTOPSY

An autopsied case of sporadic olivopontocerebellar atrophy with 'subcortical dementia' and hallucinatory paranoid state is reported. A Japanese woman without a remarkable family history showed cerebellar ataxia at the age of 61 years. followed by muscle rigidity, pyramidal signs, peripheral amyotrophy and ophthalmoplegia. She had paranoid delusion and visual and auditory hallucinations. She also developed a dementia characterized by mild memory disturbance with preservation of orientation, slowing of thought processes, affective changes including apathy and depression, impaired ability to manipulate acquired knowledge and the absence of aphasia, apraxia and agnosia. These features were consistent with those seen in subcortical dementia, first proposed by Albert. Autopsy revealed pontine tegmental atrophy in addition to a marked atrophy of the inferior olivary nucleus, pontine basis and cerebellum, in contrast to a relatively well-preserved cerebrum. The peculiar psychotic features including dementia found in the patient are discussed from a clinicopathological standpoint.