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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.
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.
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