Cerebral amyloid angiopathy related inflammation: three case reports and a review

Kong Khi Chung1, Neil E Anderson, David Hutchinson

  • 1Department of Neurology, Auckland Hospital, Auckland, New Zealand.

Insights

Cerebral amyloid angiopathy related inflammation (CAA-I) is a treatable brain condition in older adults. Prompt diagnosis and immunosuppressive therapy can lead to favorable outcomes, even without brain biopsy in some cases.

Area of Science:

  • Neurology
  • Neuroinflammation
  • Vascular Neurology

Background:

  • Cerebral amyloid angiopathy related inflammation (CAA-I), previously known by various names, is an inflammatory encephalopathy.
  • It primarily affects older adults and is characterized by specific clinical and radiological findings.

Observation:

  • This study describes three patients with histopathologically confirmed CAA-I.
  • Data from published literature are also summarized, highlighting characteristic features.

Findings:

  • Definitive diagnosis of CAA-I necessitates brain and leptomeningeal biopsy.
  • A positive response to immunosuppressive therapy is frequently observed in patients with CAA-I.

Implications:

  • Treatment for CAA-I without brain biopsy may be considered in select cases.
  • Proposed diagnostic criteria for CAA-I aim to standardize diagnosis and management.
  • Early recognition and treatment are crucial for managing this treatable encephalopathy.

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