Cerebral amyloid angiopathy with severe secondary vascular pathology: a histopathological study

Jack van Horssen, Danielle de Jong, Robert M W de Waal, Cathy Maass, Irene Otte-Holler, Berry Kremer, Marcel M Verbeek, Pieter Wesseling

Research output: Contribution to journalArticleAcademicpeer-review

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.

Original languageEnglish
Pages (from-to)321-330
Number of pages10
JournalDementia and Geriatric Cognitive Disorders
Volume20
Issue number5
DOIs
Publication statusPublished - 2005

Cite this

van Horssen, Jack ; de Jong, Danielle ; de Waal, Robert M W ; Maass, Cathy ; Otte-Holler, Irene ; Kremer, Berry ; Verbeek, Marcel M ; Wesseling, Pieter. / Cerebral amyloid angiopathy with severe secondary vascular pathology : a histopathological study. In: Dementia and Geriatric Cognitive Disorders. 2005 ; Vol. 20, No. 5. pp. 321-330.
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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.",
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Cerebral amyloid angiopathy with severe secondary vascular pathology : a histopathological study. / van Horssen, Jack; de Jong, Danielle; de Waal, Robert M W; Maass, Cathy; Otte-Holler, Irene; Kremer, Berry; Verbeek, Marcel M; Wesseling, Pieter.

In: Dementia and Geriatric Cognitive Disorders, Vol. 20, No. 5, 2005, p. 321-330.

Research output: Contribution to journalArticleAcademicpeer-review

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T2 - a histopathological study

AU - van Horssen, Jack

AU - de Jong, Danielle

AU - de Waal, Robert M W

AU - Maass, Cathy

AU - Otte-Holler, Irene

AU - Kremer, Berry

AU - Verbeek, Marcel M

AU - Wesseling, Pieter

PY - 2005

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N2 - 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.

AB - 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.

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