How is cerebral amyloid angiopathy (CAA) treated?

Updated: Dec 19, 2018
  • Author: Ravi S Menon, MD; Chief Editor: Helmi L Lutsep, MD  more...
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Answer

Answer

Cerebral amyloid angiopathy (CAA) is largely untreatable at this time. The management of CAA-related intracranial hemorrhage (ICH) is identical to the standard management of ICH. Pay special attention to the reversal of anticoagulation, the management of intracranial pressure, and the prevention of complications.

If coexisting vasculitis is found on angiography and brain biopsy, long-term treatment (up to 1y) with steroids and cyclophosphamide is indicated.

A syndrome of subacute cognitive decline, seizures, and white matter changes on MRI, with perivascular inflammatory changes on biopsy, was described, with some patients improving clinically (but not to baseline) when given corticosteroids or cyclophosphamide.

Although early investigations showed Cerebril (Neurochem, Inc), a drug developed to reduce amyloid formation and deposition, to be safe, this drug is currently not being actively studied for CAA.

An analysis of ICH subgroups in the PROGRESS (Perindopril Protection Against Recurrent Stroke Study) trial indicated that, over a follow-up period of 3.9 years, patients treated with the antihypertensive drug perindopril had a reduction in CAA-related ICHs of 77%. However, the total number of CAA-related ICHs was small (16 probable CAA-related ICHs), so definitive conclusions await larger samples. [16]


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