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- Title
An autopsied case of Down syndrome with Alzheimer pathology and α-synuclein immunoreactivity.
- Authors
Marui, W.; Iseki, E.; Kosaka, K.; Kato, M.; Adachi, Y.; Uéda, K.
- Abstract
An autopsied case of a 49-year-old man with Down syndrome is described. The patient developed mental deterioration and parkinsonism at the age of 44 years. The brain revealed Alzheimer’s disease (AD) pathology in addition to anomalies and lesions of premature senility. In the substantia nigra, many neurofibrillary tangles (NFT) and some Lewy bodies (LB) were found, suggesting that a limited degree of Parkinson’s disease (PD) pathology may combine with AD pathology to develop parkinsonism. However, spongiform change and ubiquitin-positive spheroids in the central nucleus of the amygdala, which are peculiar to dementia with Lewy bodies (DLB), were observed, suggesting that the substantia nigra lesion is similar to that of DLB rather than that of PD. In addition, α-synuclein-positive neurons in the limbic areas, predominantly in the amygdala, were found. Their incidence and distribution did not fulfill the criteria of DLB. These neurons did not show the typical features of cortical type LB, and were mostly accompanied by tau-positive NFT. These findings suggest that the presence of α-synuclein-positive neurons does not always indicate the presence of LB, and that the present case should not be regarded as the complication of DLB in Down syndrome.
- Subjects
PEOPLE with Down syndrome; ALZHEIMER'S disease; LEWY body dementia; PARKINSON'S disease
- Publication
Neuropathology, 1999, Vol 19, Issue 4, p410
- ISSN
0919-6544
- Publication type
Article
- DOI
10.1046/j.1440-1789.1999.00265.x