title: [Cerebral amyloid angiopathy and dementia].
abstract: Cerebral amyloid angiopathy (CAA) is one of the most frequent causes of intracerebral hemorrhage (ICH). The deposition of beta amyloid leads to vascular fragility due to degeneration of vessel walls, formation of microaneurysms particularly in cortical blood vessels and fibrinoid vessel wall necrosis. The Congo red positive amyloid deposits are biochemically similar to the material comprising senile plaques in Alzheimer's disease. Recurrent or multiple simultaneous hemorrhages particularly in older patients should raise the suspicion of CAA. Gradient echo magnetic resonance imaging (MRI) is a sensitive, non-invasive technique for identifying even very small hemorrhages and superficial siderosis, which may cause transient symptoms in CAA. There is also a correlation between CAA, microbleeding and cognitive decline. Inflammatory variants of CAA must be suspected whenever patients present with progressive dementia, headache and multifocal symptoms in association with CAA findings in MRI. Histopathologically, a distinction is made between CAA-related inflammation (CAA-ri) with perivascular inflammatory infiltrates and amyloid beta-related angiitis (ABRA) with histological detection of transmural vasculitis. Inflammatory variants should be treated with corticosteroids and immunosuppressants.
authors.affiliation: Klinik für Neurologie, Alfried Krupp Krankenhaus Rüttenscheid, Alfried-Krupp-Str. 21, 45131, Essen, Deutschland. peter.berlit@krupp-krankenhaus.de.
authors.forename: P
authors.initials: P
authors.lastname: Berlit
authors.affiliation: Institut für Neuropathologie, Universitätskinikum Essen, Essen, Deutschland.
authors.forename: K
authors.initials: K
authors.lastname: Keyvani
authors.affiliation: Klinik für Neurologie, Alfried Krupp Krankenhaus Rüttenscheid, Alfried-Krupp-Str. 21, 45131, Essen, Deutschland.
authors.forename: M
authors.initials: M
authors.lastname: Krämer
authors.affiliation: Klinik für Neurologie, Alfried Krupp Krankenhaus Rüttenscheid, Alfried-Krupp-Str. 21, 45131, Essen, Deutschland.
authors.forename: R
authors.initials: R
authors.lastname: Weber
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D016657:Cerebral Amyloid Angiopathy / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*; D003704:Dementia / Q000175:diagnosis* / Q000209:etiology / Q000517:prevention & control*; D003937:Diagnosis, Differential; D019317:Evidence-Based Medicine; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging / Q000379:methods; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents
keywords: Cortical vessels; Degenerative vascular disease; Immunosuppressants; Intracerebral hemorrhage; Microbleeding