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.
MeSH terms
-
Adult
-
Aged
-
Aged, 80 and over
-
Alzheimer Disease / pathology
-
Anti-Inflammatory Agents / therapeutic use
-
Blood Vessels / pathology
-
Brain / pathology
-
Cerebral Amyloid Angiopathy / drug therapy
-
Cerebral Amyloid Angiopathy / pathology*
-
Cerebral Cortex / pathology
-
Cyclophosphamide / therapeutic use
-
Dexamethasone / therapeutic use
-
Fatal Outcome
-
Female
-
Headache / etiology
-
Humans
-
Immunosuppressive Agents / therapeutic use
-
Inflammation / pathology*
-
Magnetic Resonance Imaging
-
Male
-
Methylprednisolone / therapeutic use
-
Middle Aged
-
Paresis / etiology
-
Prognosis
-
Seizures / etiology
-
Tomography, X-Ray Computed
Substances
-
Anti-Inflammatory Agents
-
Immunosuppressive Agents
-
Dexamethasone
-
Cyclophosphamide
-
Methylprednisolone