We clinically studied 4 patients with Joseph disease in a Japanese family, who developed mild dementia, and neuropathologically examined one autopsied case among them. Neurological findings included cerebellar ataxia, progressive external ophthalmoplegia, peripheral amyotrophy, and pyramidal signs. Mental state examination revealed mild impairment of intelligence. The dementia was 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. CT demonstrated severe atrophy of the brain stem and severe dilatation of the fourth ventricle with mild atrophy of the cerebral and cerebellar hemispheres. EEG showed slowing of background activity. The autopsied case showed atrophy of the tegmentum of the brain stem in addition to the common pathological findings of Joseph disease. The features of dementia in the 4 patients were similar to those seen in ‘subcortical dementia’, suggesting that its pathological basis may be involvement of the tegmentum of the brain stem.

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