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.
The subject presented with intellectual decline followed by progressive muscle weakness of the bilateral upper limbs when he was 60 years old. He had a point mutation (methionin-valine) at 129 prion protein codon. He died at the age of 63 and necropsy revealed bilateral frontal lobe atrophy. The frontal cortex showed neuronal cell loss in layers II and III with spongiform change. Reusche silver impregnation technique for β-peptide combined with ubiquitin immunostaining revealed perineuronal structures encircling degenerated neurons and ubiquitin-immunoreactive (IR) dot-like deposits. They were distributed particularly in the temporal neocortex and entorhinal cortex. They differed from either classic senile or diffuse plaque by the absence of amyloid core in the center and of amyloid fibrils. Ubiquitin-IR materials were also found as neuronal inclusions in the hippocampal granular cells. Nigral degeneration and neuronal loss in the hypoglossal nerve nucleus and in the anterior horn of the spinal cord were also found and spinal cord motoneurons had Bunina body inclusions. The clinical features and pathological findings were consistent with non-Alzheimer dementia with status spongiosus and neuronal cell loss. The unusual perineuronal structures found in our case might be a specific cellular pathology of dementia of the frontal lobe type.
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