To elucidate the influence of the alpha1-antichymotrypsin (ACT) polymorphism on Alzheimer-type neuropathological changes and the development of sporadic Alzheimer's disease (AD), we studied the relationship between the ACT polymorphism and the severity of Alzheimer-type neuropathological changes in the brains from AD patients and nondemented subjects. There was no association of the ACT polymorphism with Alzheimer-type neuropathological changes in AD or nondemented individuals. ACT polymorphism was not associated with the development of AD. These results remained nonsignificant when the ACT genotype groups were divided into subgroups according to the apolipoprotein E (ApoE) epsiolon4 status. Our study shows that the ACT polymorphism has no effect on Alzheimer-type neuropathological changes or the development of AD, either alone or in combination with ApoE epsiolon4.

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Senile dementia of the neurofibrillary tangle type: a comparison with Alzheimer's disease.

A subset of senile dementia, 'senile dementia (SD) of the neurofibrillary tangle (NFT) type' (SD-NFT), is characterized by numerous NFTs in the hippocampal region and absence or scarcity of senile plaques throughout the brain. To elucidate the pathogenesis of SD-NFT in comparison with Alzheimer's disease (AD), we investigated the hippocampal lesions and analyzed the tau gene. The hippocampal regions from 5 patients with SD-NFT were neuropathologically evaluated in comparison with AD and nondemented control subjects. The tau gene was analyzed in 3 patients with SD-NFT. The densities of NFTs in the CA1/subiculum and entorhinal cortex of SD-NFT were significantly higher than those in AD. However, hippocampal atrophy, neuronal and synaptic loss, and astrocytic and microglial proliferation in SD-NFT were significantly mild compared with AD. There was no significant difference between SD-NFT and AD in the immunoreactivities of NFTs with different anti-tau antibodies. No mutation was found in the tau gene from the SD-NFT patients. Our results indicate that the neurodegenerative process with NFT formation of the hippocampal region in SD-NFT would be different from that in AD.

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Vascular variant of Alzheimer's disease characterized by severe plaque-like beta protein angiopathy.

In the neuropathological investigations of 32 demented patients with severe cerebral amyloid angiopathy (CAA), we found an unusual case, a 90-year-old woman, presenting with the pathology of atypical Alzheimer's disease (AD). The case showed severe plaque-like angiopathy, in which all the senile plaques except for some diffuse plaques were formed around amyloid beta (/A4) protein-laden vessels. Analysis of the amyloid beta protein precursor gene from the patient revealed no mutation. Our results have indicated that, among demented patients with severe CAA, there is the vascular variant of AD characterized by severe plaque-like beta protein angiopathy.

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