Abstract
Cerebral amyloid angiopathy (CAA) is the deposition of amyloid in the wall of intracranial blood vessels, and leads to the appearance of hemorrhage, ischemia or leukoencephalopathy. The clinical manifestations of the CAA are highly variable, such as cognitive impairment, behavioral abnormalities, focal neurological deficits, headache or seizures. A subtype, Cerebral amyloid angiopathy-related inflammation (CAA-RI) has recently been reported in the world literature. We report the case of a 74-year-old with a rapidly progressive dementia about three months duration, associated with headache, meningismus, decreased strength in right hemisphere, multiple parenchymal hemorrhagic lesions, diffuse hemosiderosis, cerebral edema focal and histological study with evidence of intracerebral amyloid.
The diagnosis of CAA is based on a clinical history compatible, neuroimaging showing hemosiderosis or multiple bleeding, predominantly in posterior fossa, and in some cases histological examination to confirm the presence of amyloid in intracranial microcirculation. Modified Boston criteria, unify the findings for the diagnosis of CAA, with varying degrees of certainty. Sometimes, as in the case presented, the CAA is associated with an inflammatory component, and is manifested by a rapidly progressive dementia, becoming a true diagnostic challenge.
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