Cerebral amyloid angiopathy with dementia: clinicopathological studies of 17 cases

To study clinicopathologically 17 cases of cerebral amyloid angiopathy (CAA) with dementia and to investigate the possible neuropathology of this disorder.

Of 362 autopsy cases, 17 who had had dementia with proved CAA were studied. Hematoxylin and eosin, Congo red, Bodian and beta/A4 protein immunohistochemical stain were used. Brain sections were observed under light microscopy and polarized light microscopy. The amyloid was confirmed by positive Congo red stain with apple-green birefringence under polarized light and positive beta/A4 immunohistochemical stain. The severity of amyloid deposit was graded as slight, moderate and severe.

In the 17 cases of CAA associated with dementia, Alzheimer's disease was found in 3 cases, Pick's disease in 1, multi-infarct dementia in 6, multi-hemorrhage dementia in 4, and pure CAA in 3.

CAA not only is closely related to Alzheimer's disease, but also causes cortical multiple small infarcts and hemorrhagic lesions, which lead to dementia. Moreover, pure CAA may produce dementia through hypoperfusion of the cortex by CAA-related hyalinization and stenosis of the arteries, which lead to the loss of cortical neurons.