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  • Updated 07.05.2023
  • Released 05.16.1997
  • Expires For CME 07.05.2026

Cerebral amyloid angiopathy



Cerebral amyloid angiopathy is recognized as a major cause of intracerebral hemorrhage in the elderly, as well as an important contributor to vascular cognitive impairment and dementia. The following are advances highlighted in the article: (1) the updated Boston criteria version 2.0 for the diagnosis of cerebral amyloid angiopathy, (2) the pathophysiological framework of cerebral amyloid angiopathy progression, and (3) the potential transmissibility of cerebral amyloid angiopathy through cadaveric dura matter exposure.

Key points

• Cerebral amyloid angiopathy is a common neuropathology in the aging brain, most often recognized in clinical practice as the cause of multiple, strictly lobar intracerebral hemorrhages, microbleeds, or superficial sulcal/meningeal bleeding.

• Cerebral amyloid angiopathy-related microbleeds and superficial sulcal/meningeal bleeding are sensitively detected by T2*-weighted gradient-echo or susceptibility-weighted imaging MRI techniques.

• Advanced cerebral amyloid angiopathy is also associated with nonhemorrhagic brain lesions, such as white matter T2-hyperintensities, especially with a multifocal subcortical pattern, enlarged perivascular spaces in the centrum semiovale, and cortical microinfarcts.

• The presence of cortical superficial siderosis is the strongest risk factor for subsequent lobar intracerebral hemorrhage in patients with cerebral amyloid angiopathy.

• Reasonable steps for limiting the risk of recurrent cerebral amyloid angiopathy–related hemorrhage are blood pressure control, avoidance of anticoagulation in patients with biomarkers proven to convey high risk, and withholding of other antithrombotics in the absence of clear-cut indication.

• A subset of cerebral amyloid angiopathy presents with subacute mental status changes, headache, seizures, and amyloid-related vascular inflammation, related in at least some instances to anti-amyloid autoantibodies in the cerebrospinal fluid and often improving with immunosuppressive treatment.

Historical note and terminology

Cerebral amyloid angiopathy has been recognized since the early part of the 20th century. In the German literature in 1907 and 1909, Fischer and Oppenheim described cases that, in retrospect, probably were cerebral amyloid angiopathy (64). Early labels for this clinicopathologic condition included “drusige Entartung der Artien und Kapillaren” and “amyloidose cerebrale et meningee.” In 1927, Divry described amyloid in vessel walls and neuritic plaques, based on birefringence when viewed under polarized light with Congo red staining, leading to the term “congophilic angiopathy.” In 1952, Morel and Wildi coined the term “dyshoric angiopathy” to describe vascular amyloid that invades the surrounding parenchyma. In 1935, the occurrence of a familial form of cerebral amyloid angiopathy in Iceland was described by Arnason and later labeled “hereditary cerebral hemorrhage with amyloid—Icelandic type” (04). A second familial form was recognized in the Netherlands in 1964 by Luyendijk and later labeled “hereditary cerebral hemorrhage with amyloid—Dutch type.” Although an association of cerebral amyloid angiopathy with Alzheimer disease was recognized in the 1940s, it was not until the 1970s that Jellinger and others realized that cerebral amyloid angiopathy is an integral part of Alzheimer disease (95). Also in the 1970s, several groups suggested a relation between cerebral amyloid angiopathy and nontraumatic intracerebral hemorrhage. Perhaps the most pivotal event in the history of cerebral amyloid occurred in 1984 when Glenner and Wong isolated and partially sequenced the amyloid protein found in meningeal arteries of brains of patients with Alzheimer disease and Down syndrome (67). In 1987, Pardridge and colleagues isolated and partially sequenced the same amyloid peptide from intraparenchymal arterioles in patients with Alzheimer disease (143). Currently, more than 10 central nervous system diseases have been associated with various forms of cerebral amyloid angiopathy. Over the last decades, interest in cerebral amyloid angiopathy has escalated, spurred by the development of molecular biological techniques and neuroimaging methods to dissect the cerebral amyloidoses, including cerebral amyloid angiopathy–related hemorrhage and Alzheimer disease.

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