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Moyamoya Syndrome

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NORD is very grateful to R. Michael Scott, MD, Neurosurgeon-in-Chief, The Children's Hospital, Boston, and Edward R. Smith, MD, Department of Neurosurgery, Children's Hospital Boston/Harvard Medical School, for assistance in the preparation of this report.

Synonyms of Moyamoya Syndrome
  • moyamoya disease

Disorder Subdivisions



General Discussion
Moyamoya syndrome is a progressive disorder that affects the blood vessels in the brain (cerebrovascular). It is characterized by the narrowing (stenosis) and/or closing (occlusion) of the carotid artery inside the skull, the major artery that delivers blood to the brain. At the same time, tiny blood vessels at the base of the brain open up in an apparent attempt to supply blood to the brain distal to the blockage. These tiny vessels are the "moyamoya" vessels for which the disease was named. Inadequate blood supply then leads to reduced oxygen delivery to the brain, and it is this oxygen deprivation that causes the signs of moyamoya. One of the symptoms is typically stroke, which results in paralysis of the face, arms or legs, loss of speech, etc., or temporary loss of neurologic function of body parts or speech (transient ischemic attacks). Other symptoms that may result include headaches, visual disturbances, developmental delay, and seizures. Approximately 10% of cases of moyamoya in Asian countries have a genetic cause. Patients with this arteriopathy that occurs either on a familial or idiopathic basis are said to have moyamoya disease. Patients in whom the artery changes occur in association with another process such as sickle cell disease or Down syndrome are said to have moyamoya syndrome. In this report, we use the term "moyamoya syndrome" as a shorthand for both forms.
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Organizations related to Moyamoya Syndrome
  • Genetic and Rare Diseases (GARD) Information Center
    PO Box 8126
    Gaithersburg MD 20898-8126
    Phone #: 301-251-4925
    800 #: 888-205-2311
    e-mail: http://rarediseases.info.nih.gov/GARD/EmailForm.aspx
    Home page: http://rarediseases.info.nih.gov/GARD
  • MUMS National Parent-to-Parent Network
    150 Custer Court
    Green Bay WI 54301-1243
    Phone #: 920-336-5333
    800 #: 877-336-5333
    e-mail: mums@netnet.net
    Home page: http://www.netnet.net/mums/
  • Madisons Foundation
    PO Box 241956
    Los Angeles CA 90024
    Phone #: 310-264-0826
    800 #: N/A
    e-mail: getinfo@madisonsfoundation.org
    Home page: http://www.madisonsfoundation.org
  • Moyamoya.com
    PO Box 9602
    Wichita KS 67277
    Phone #: N/A
    800 #: N/A
    e-mail: admin@moyamoya.com
    Home page: http://www.moyamoya.com
  • National Institute of Neurological Disorders and Stroke (NINDS)
    P.O. Box 5801
    Bethesda MD 20824
    Phone #: 301-496-5751
    800 #: 800-352-9424
    e-mail: me20t@nih.gov
    Home page: http://www.ninds.nih.gov/

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Last modified Wednesday, November 26, 2008