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Research paper
Cerebral amyloid angiopathy related inflammation: three case reports and a review
  1. Kong Khi Chung1,
  2. Neil E Anderson1,
  3. David Hutchinson1,
  4. Beth Synek2,
  5. P Alan Barber1,3
  1. 1Department of Neurology, Auckland Hospital, Auckland, New Zealand
  2. 2Department of Forensic Pathology, Auckland Hospital, Auckland, New Zealand
  3. 3Faculty of Medicine and Health Sciences, University of Auckland, Auckland, New Zealand
  1. Correspondence to Professor A Barber, Department of Medicine, Faculty of Medicine and Health Sciences, University of Auckland, Private Bag 92019, Auckland 1142, New Zealand; a.barber{at}auckland.ac.nz

Abstract

Cerebral amyloid angiopathy related inflammation (CAA-I), previously described under various names, is a treatable encephalopathy usually occurring in older adults. Here, three patients are described with histopathologically confirmed CAA-I, and summarised data from the published literature are presented. CAA-I has a characteristic combination of clinical and radiological features. Definite diagnosis requires brain and leptomeningeal biopsy. A favourable response to immunosuppressive therapy is common and treatment without brain biopsy may be considered in selected patients. Diagnostic criteria for CAA-I are proposed.

  • Cerebrovascular disease

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Footnotes

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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