Medication Summary
Drug therapy for moyamoya disease depends on the particular manifestations of the disease. For hemorrhage, therapy revolves around the management of hypertension (if present).
For ischemic stroke, anticoagulation with heparin or warfarin may be considered. Safety and efficacy have not been fully established for these drugs, and careful analysis of risk and benefits is needed. These drugs could be useful if thrombosis of vessels is present, but they do not alter the natural history of the disease and they considerably increase the risk of hemorrhage with large strokes.
The same considerations are true for aspirin and other antiplatelet agents. Treatment with anticoagulation or antiplatelet agents should be pursued only after consultation with a neurologist who is experienced in stroke management.
Anticoagulants, Hematologic
Class Summary
These agents are given for the prevention of further thrombosis and potential infarction of the brain. Caution: Anticoagulation is of unproven benefit in ischemic stroke associated with moyamoya disease. This therapy is therefore considered to be empirical.
Heparin
Heparin is administered intravenously; it is frequently given with initial bolus in cardiac situations. In stroke, bolus is not recommended. The target dose is aimed at maintaining an activated partial thromboplastin time (aPTT) of 1.5-2 times control. A computed tomography (CT) scan of the brain must be done prior to any anticoagulant use to rule out preexisting intracranial hemorrhage.
Warfarin (Coumadin, Jantoven)
Warfarin, which is administered orally, is used if long-term anticoagulation is needed. The international normalized ratio (INR) is followed, with a target range of 2-3. A CT scan of the brain must be done prior to any anticoagulant use to rule out preexisting intracranial hemorrhage.
Antiplatelet agents
Class Summary
These agents can be considered to help prevent future ischemic strokes. As with anticoagulation, aspirin is of unproven benefit in moyamoya disease; its use is considered empirical.
Aspirin (Ecotrin, Ascriptin Maximum Strength, Ascriptin, Bayer Aspirin)
Aspirin's efficacy in preventing stroke relies on the inhibitory effect of aspirin on platelet function. This presumably helps to prevent thrombus formation and propagation.
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Schematic representation of the circle of Willis, arteries of the brain, and brain stem.
