title: Cerebral amyloid angiopathy with severe secondary vascular pathology: a histopathological study.
abstract: Cerebral amyloid angiopathy (CAA) is a common neuropathological finding and is characterized by deposition of fibrillar amyloid in cortical and leptomeningeal vessels. In this study we describe the macroscopic and microscopic neuropathological findings of 5 patients with severe CAA-associated secondary vascular changes, including smooth muscle cell degeneration, hyalinization, 'double-barreling' phenomenon, macrophage infiltration, and aneurysmal dilatation of the vessel wall. In 3 of the 5 patients these vascular changes were associated with multiple small hemorrhages, whereas in 2 patients areas of ischemic necrosis were observed. However, none of these patients suffered from large (lobar) hemorrhagic accidents. Nevertheless, severe CAA, particularly when associated with secondary vascular pathology, may lead to vascular dementia-like ischemic changes. Hence, the distinction between patients with severe CAA and secondary vascular abnormalities from those suffering from vascular dementia can be difficult. We speculate that CAA, particularly when associated with secondary vascular pathology, although not resulting in large hemorrhages, may contribute to cognitive decline. The functional impact of CAA and CAA-related secondary vascular changes on cognitive performance warrants further exploration.
authors.affiliation: Department of Pathology, University Medical Center, Nijmegen, The Netherlands.
authors.forename: Jack
authors.initials: J
authors.lastname: van Horssen
authors.forename: Danielle
authors.initials: D
authors.lastname: de Jong
authors.forename: Robert M W
authors.initials: RM
authors.lastname: de Waal
authors.forename: Cathy
authors.initials: C
authors.lastname: Maass
authors.forename: Irene
authors.initials: I
authors.lastname: Otte-Holler
authors.forename: Berry
authors.initials: B
authors.lastname: Kremer
authors.forename: Marcel M
authors.initials: MM
authors.lastname: Verbeek
authors.forename: Pieter
authors.initials: P
authors.lastname: Wesseling
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D016229:Amyloid beta-Peptides / Q000378:metabolism; D016564:Amyloid beta-Protein Precursor / Q000235:genetics; D001057:Apolipoproteins E / Q000235:genetics; D016657:Cerebral Amyloid Angiopathy / Q000235:genetics / Q000473:pathology*; D002536:Cerebral Arteries / Q000473:pathology; D002560:Cerebrovascular Circulation / Q000502:physiology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007249:Inflammation / Q000473:pathology; D008297:Male; D019636:Neurodegenerative Diseases / Q000473:pathology; D012307:Risk Factors
chemical_list: D016229:Amyloid beta-Peptides; D016564:Amyloid beta-Protein Precursor; D001057:Apolipoproteins E