A clinical and pathological study of a Japanese case of XXXX /Parkinsonism-Dementia Complex with family history.

@entity1 @entity304 @entity146 @entity303 @entity2655 @entity39348 @entity1399 @entity3072 @entity194 @entity746
This report concerns a Japanese family with neuropathological findings consistent with @entity746 / @entity304 - @entity303 complex ( @entity2655 /PDC) in the Island of Guam. The proband was a 68-year-old @entity1 with an 8-year history of @entity304 which was followed by @entity146 symptoms and @entity39348 5 years after the onset. She had a family history of @entity304 associated with @entity303 in all of her three siblings. They grew up in the Hobara village, a focus of @entity746 in the Kii Peninsula of Japan in their childhood. Their parents were not consanguineous nor natives of the Kii Peninsula. The brain weight was 1040 g and there were mild frontal lobe @entity194 , moderate @entity194 of pes hippocampi, decoloration of the substantia nigra and locus coeruleus, and @entity194 of the anterior root of the spinal cord. The microscopic examinations revealed degeneration of @entity1399 portion of the hippocampus to the parahippocampus gyrus, substantia nigra, locus coeruleus and spinal anterior horn with Bunina bodies. The spinal pyramidal tracts also mildly degenerated. @entity3072 ( @entity3072 ) were observed in the cerebral cortex, especially in the cortices from hippocampus to lateral occipitotemporal gyri, basal nucleus of Mynert, basal ganglia, thalamus, substantia nigra and widespread regions of the central nervous system through the brainstem to spinal cord including the nucleus of Onufrowitcz. In spite of a small amount of the senile plaques in the cerebral cortex and Lewy bodies in the substantia nigra and locus coeruleus, abundant @entity3072 were distributed mainly in the third layer of the cerebral cortex, which is the characteristic feature of @entity2655 /PDC. Thus, this was likely to be an @entity2655 /PDC case outside the Guam Island. A tau mutation was not found on DNA analysis.
