Sporadic Olivopontocerebellar Atrophy with 'Subcortical Dementia' and Hallucinatory Paranoid State: Report of an Autopsy

Abstract

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.

Striatonigral Degeneration Combined with Olivopontocerebellar Atrophy with Subcortical Dementia and Hallucinatory State

“…Therefore, we think that the development of the auditory hallucinations may be attributable to the disease process rather than to a side effect of the drugs. No report has described the hallucina tions in SND except for Adams's case showing hallucina tions due to antiparkinsonian drugs [2], Our present case is reminiscent of our recently published case of OPCA with brain stem tegmental atrophy [11]. The case of OPCA showed subcortical dementia and a hallucinatory paranoid state.…”

“…Although the spinal cord under the level of the C2 segment was not available, the orthostatic hypotension found in the present case, which is a main feature of SDS [5], may reflect pathological changes in the intermediolateral col umns. In addition, our case revealed brain stem tegmental atrophy which might have been overlooked or paid little attention of OPCA [11],…”