NONFAMILIAL OLIVOPONTOCEREBELLAR ATROPHY COMBINED WITH LATE-ONSET ALZHEIMERS-DISEASE - A CLINICOPATHOLOGICAL CASE-REPORT

A 76-year-old woman with olivopontocerebellar atrophy (OPCA) presented with progressive intellectual deterioration. She showed cerebellar at axia and muscle atrophy and weakness, and gradually developed generali zed dementia with visuospatial disturbance. An autopsy revealed numero us senile plaques (SPs), neurofibrillary tangles (NFTs) and neuropil t hreads particularly in the CA1, subiculum and entorhinal cortex and to a lesser degree in the cerebral neocortex shown by immunostaining and specific silver impregnation techniques. The nucleus basalis of Meyne rt had numerous NFTs with fibrillary gliosis and neuronal cell loss. T he basis pontis was markedly atrophied and the pontine nucleus had sev ere neuronal depopulation and gliosis. The pontine transverse fibers w ere demyelinated with their axons being fragmented, The cerebellar whi te matter was also severely degenerated. The striatum, Onuf's and inte rmediolateral nuclei of the spinal cord remained unchanged. Ubiquitin immunohistochemistry and Gallyas silver impregnation technique reveale d oligodendroglial inclusions In the pontine nucleus, corticopontine t ract, cerebral and cerebellar white matter. On double immunostaining o f KP1 and ubiquitin, globular neurite SPs encircled by KP1-positive fi brous structures were found in the hippocampus and cerebral neocortex. The curly neurite SPs contained KP1-positive granules. The KP1-positi ve microglial cells were distributed widely in the cerebral white matt er and HLA-DR-positive ones were found around the SPs. The present cas e showed generalized dementia compatible with Alzheimer's disease (AD) and had a pathologically limbic type of late onset AD. This is the fi rst case where AD affected non-familial OPCA. (C) 1998 Elsevier Scienc e B.V.