Medscape is available in 5 Language Editions – Choose your Edition here.


Moyamoya Disease Medication

  • Author: Roy Sucholeiki, MD; Chief Editor: Amy Kao, MD  more...
Updated: Apr 15, 2016

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 is administered intravenously; it is frequently given with initial bolus in cardiac situations. In stroke, bolus 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.

Contributor Information and Disclosures

Roy Sucholeiki, MD Director, Comprehensive Seizure and Epilepsy Program, The Neurosciences Institute at Central DuPage Hospital

Roy Sucholeiki, MD is a member of the following medical societies: American Academy of Neurology, American Neuropsychiatric Association, American Epilepsy Society

Disclosure: Nothing to disclose.


Jasvinder Chawla, MD, MBA Chief of Neurology, Hines Veterans Affairs Hospital; Professor of Neurology, Loyola University Medical Center

Jasvinder Chawla, MD, MBA is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD Attending Neurologist, Children's National Medical Center

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, Child Neurology Society

Disclosure: Have stock from Cellectar Biosciences; have stock from Varian medical systems; have stock from Express Scripts.


Robert Stanley Rust Jr, MD, MA Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia; Chair-Elect, Child Neurology Section, American Academy of Neurology

Robert Stanley Rust Jr, MD, MA is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, American Neurological Association, Child Neurology Society, International Child Neurology Association, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

  1. Janda PH, Bellew JG, Veerappan V. Moyamoya disease: case report and literature review. J Am Osteopath Assoc. 2009 Oct. 109(10):547-53. [Medline].

  2. Mineharu Y, Takenaka K, Yamakawa H, et al. Inheritance pattern of familial moyamoya disease: autosomal dominant mode and genomic imprinting. J Neurol Neurosurg Psychiatry. 2006 Sep. 77(9):1025-9. [Medline].

  3. Mineharu Y, Liu W, Inoue K, Matsuura N, Inoue S, Takenaka K. Autosomal dominant moyamoya disease maps to chromosome 17q25.3. Neurology. 2008 Jun 10. 70(24 Pt 2):2357-63. [Medline].

  4. Kamada F, Aoki Y, Narisawa A, Abe Y, Komatsuzaki S, Kikuchi A, et al. A genome-wide association study identifies RNF213 as the first Moyamoya disease gene. J Hum Genet. 2011 Jan. 56(1):34-40. [Medline].

  5. Ma J, Liu Y, Ma L, Huang S, Li H, You C. RNF213 polymorphism and Moyamoya disease: A systematic review and meta-analysis. Neurol India. 2013 Jan-Feb. 61(1):35-9. [Medline].

  6. Kim SJ, Heo KG, Shin HY, Bang OY, Kim GM, Chung CS. Association of thyroid autoantibodies with moyamoya-type cerebrovascular disease: a prospective study. Stroke. 2010 Jan. 41(1):173-6. [Medline].

  7. Im SH, Oh CW, Kwon OK, et al. Moyamoya disease associated with Graves disease: special considerations regarding clinical significance and management. J Neurosurg. 2005 Jun. 102(6):1013-7. [Medline].

  8. Uchino K, Johnson A, Claiborne S, Tirschwell DL. Moyamoya disease in Washington State and California. Neurology. 2005. 65:956-958. [Medline].

  9. Kim JS. Moyamoya Disease: Epidemiology, Clinical Features, and Diagnosis. J Stroke. 2016 Jan. 18 (1):2-11. [Medline].

  10. Li H, Zhang ZS, Dong ZN, et al. Increased Thyroid Function and Elevated Thyroid Autoantibodies in Pediatric Patients With Moyamoya Disease: A Case-Control Study. Stroke. 2011 Feb 24. [Medline].

  11. Kim T, Oh CW, Bang JS, Kim JE, Cho WS. Moyamoya Disease: Treatment and Outcomes. J Stroke. 2016 Jan. 18 (1):21-30. [Medline].

  12. Starke RM, Komotar RJ, Hickman ZL, et al. Clinical features, surgical treatment, and long-term outcome in adult patients with moyamoya disease. Clinical article. J Neurosurg. 2009 Nov. 111(5):936-42. [Medline]. [Full Text].

  13. Fung LW, Thompson D, Ganesan V. Revascularisation surgery for paediatric moyamoya: a review of the literature. Childs Nerv Syst. 2005 May. 21(5):358-64. [Medline].

  14. Scott RM, Smith JL, Robertson RL, et al. Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg. 2004 Feb. 100(2 Suppl Pediatrics):142-9. [Medline].

  15. Hoffman HJ. Moyamoya disease and syndrome. Clin Neurol Neurosurg. 1997 Oct. 99 Suppl 2:S39-44. [Medline].

  16. Cohen N, Berant M, Simon J. Moyamoya and Fanconi's anemia. Pediatrics. 1980 Apr. 65(4):804-5. [Medline].

  17. Gosalakkal JA. Moyamoya disease: a review. Neurol India. 2002 Mar. 50(1):6-10. [Medline].

Schematic representation of the circle of Willis, arteries of the brain, and brain stem.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.