What is the management and treatment of amyloid angiopathy?

Management and Treatment of Cerebral Amyloid Angiopathy

Immediate Management of Acute Intracerebral Hemorrhage

For patients presenting with CAA-related intracerebral hemorrhage, target systolic blood pressure to 130-150 mmHg immediately and permanently discontinue all anticoagulation and antiplatelet agents. 1

• Initiate aggressive BP lowering as soon as CAA-related hemorrhage is identified to reduce hematoma expansion and cerebral edema 1
• Provide multidisciplinary stroke unit care with early rehabilitation 1
• Surgical evacuation is appropriate only for life-threatening mass effect threatening herniation, as it has not been shown to improve survival and carries risk of recurrent hemorrhage 1

Long-Term Blood Pressure Management

Strict blood pressure control is the only proven modifiable intervention to reduce CAA-related hemorrhage recurrence. 1

• Maintain long-term BP target <140/90 mmHg (or <130/80 mmHg with diabetes or chronic kidney disease) 1
• This is critical because recurrence rates are 2.1-3.7% per patient-year, substantially higher in lobar locations 1

Antithrombotic Management Strategy

Permanently avoid all anticoagulation and antiplatelet therapy in patients with diagnosed CAA due to extremely high risk of recurrent lobar hemorrhage. 1

• For CAA patients with atrial fibrillation requiring stroke prevention, left atrial appendage occlusion is the preferred strategy over oral anticoagulation 1
• This recommendation applies regardless of CHA₂DS₂-VASc score 1
• CAA must be diagnosed using validated clinico-radiological criteria (Boston criteria) before making these anticoagulation decisions 1, 2

Diagnostic Confirmation

MRI is superior to CT for identifying CAA and should include T2-weighted gradient-echo sequences to detect characteristic features.* 1

• Look for lobar microbleeds, cortical superficial siderosis, and white matter changes on MRI 1
• Absence of hypertensive hemorrhage patterns (basal ganglia, thalamus, pons, cerebellum) suggests CAA 1
• The diagnosis is based on validated clinico-radiological Boston criteria combining clinical presentation with imaging findings 1, 2

Risk Stratification for Recurrent Hemorrhage

Identify high-risk features that predict recurrence:

• Lobar ICH location is the strongest predictor of recurrence 1
• Older age increases risk 1
• Apolipoprotein E ε2 or ε4 alleles confer genetic susceptibility 1
• Greater number of microbleeds on T2*-weighted gradient-echo MRI 1
• Previous hemorrhage history 1
• Presence of cortical superficial siderosis 1

Management of CAA-Related Inflammation

For the minority of patients presenting with CAA-related inflammation (characterized by subacute cognitive decline, seizures, or headaches with white matter changes), immunosuppressive treatment is often responsive in the acute phase. 3

• This represents a distinct inflammatory variant requiring separate diagnostic and treatment approach 3
• Consider immunosuppression when inflammatory features are present 3

Current Limitations and Future Directions

No effective therapeutics currently exist to cure or halt CAA progression, making prevention of hemorrhage through BP control and avoidance of antithrombotics the cornerstone of management. 1, 3

• Iron chelating agents (deferoxamine) are in early phase trials for oxidative injury after ICH 1
• Anti-amyloid therapies show early promise in animal models but require human testing 1
• Clinical trials are ongoing for NOACs and left atrial appendage occlusion in ICH survivors 1

Common Pitfalls to Avoid

• Do not restart anticoagulation or antiplatelet therapy even for compelling indications like atrial fibrillation—use left atrial appendage occlusion instead 1
• Do not pursue surgical evacuation routinely—reserve only for life-threatening mass effect 1
• Do not overlook MRI imaging—CT alone is insufficient for diagnosis and risk stratification 1
• Do not use standard hypertensive ICH management protocols—CAA requires specific BP targets and long-term control strategies 1