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The Inflammatory Form of Cerebral Amyloid Angiopathy or “Cerebral Amyloid Angiopathy-Related Inflammation” (CAARI)

  • Howard S. KirshnerEmail author
  • Michael Bradshaw
Behavior (HS Kirshner, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Behavior

Abstract

Cerebral amyloid angiopathy-related inflammation (CAARI) is a recently recognized syndrome of reversible encephalopathy seen in a subset of patients with cerebral amyloid angiopathy (CAA). CAA is a disorder of the elderly in which amyloid peptides are deposited in the walls of cerebral arteries, leading to microhemorrhages, macrohemorrhages, and eventually dementia. In a few cases, the amyloid deposition is accompanied by inflammation or edema. The clinical syndrome of CAARI is distinguished by subacute neurobehavioral symptoms, headaches, seizures, and stroke-like signs, contrasting the acute intracranial hemorrhage typically seen in CAA. Magnetic resonance imaging findings may be symmetric or asymmetric and involve patchy or confluent T2 hyperintense lesions in the cortex and subcortical white matter. Recent diagnostic criteria have been proposed which help distinguish CAARI from alternative diagnoses. Improvement has been reported in most cases with immunosuppression, although a few cases have had recurrent symptoms. Here, we review the clinical and radiologic features of CAARI and compare these with CAA.

Keywords

Amyloid beta peptides Cerebral amyloid angiopathy CAARI Gradient echo MR imaging Microhemorrhages or microbleeds 

Definition of Terms

Amyloid beta peptides

generally 40 or 42 amino acid peptides, which can bind together into beta-pleated sheets to form deposits of amyloid

Cerebral amyloid angiopathy

the condition of amyloid deposition in cerebral arteries

CAARI

cerebral amyloid angiopathy-related inflammation

Gradient echo MR imaging

a sequence in which blood products “bloom” and become easily visible on MRI

Microhemorrhages or microbleeds

punctuate hemorrhages ≤5 mm, usually seen in the posterior areas of the brain, near the cortical/subcortical junction in cerebral amyloid angiopathy

Notes

Compliance with Ethics Guidelines

Conflict of Interest

The authors declare that they have no competing interests.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Department of NeurologyVanderbilt University Medical CenterNashvilleUSA
  2. 2.Vanderbilt University Medical CenterNashvilleUSA

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