Moyamoya is a rare, cerebrovascular disorder caused by the progressive blockage (occlusion) of the large branches of the internal carotid artery at the base of the brain in an area called the "circle of Willis".
To feed the starved cerebral tissue caused by Moyamoya disease, the body forms a network of dilated and weakened (friable) blood vessels. The blood vessels resemble a "puff of smoke" on diagnostic cerebral angiography tests. Originally identified in Japanese patients, this condition was named "moyamoya," the Japanese word for the "puff of smoke" appearance. Moyamoya disease is most commonly seen in children and young adults and is typically diagnosed in children 10 years of age and younger, as well as in adults (more commonly women) in their 30's.
The Moyamoya Center at North Shore-LIJ Health System's renowned Cushing Neuroscience Institute is a multidisciplinary group dedicated to the diagnosis, treatment and support of patients and families with Moyamoya disease and other neurosurgical and neurological conditions. Members of the team include a vascular neurologist, pediatric neurologist, neurosurgeon, endovascular specialists, neuroradiologists, neuroscience nurses and physician assistants. Our center works closely with the neurological specialists at Cushing Neuroscience Institute's Stroke Center and the Brain Aneurysm Center.
The appearance of Moyamoya disease on diagnostic imaging tests may be seen in association with other medical conditions. When the changes in the brain blood vessels are associated with another underlying medical problem, the condition is termed Moyamoya syndrome. Medical conditions associated with Moyamoya syndrome include:
- Sickle cell anemia
- Neurofibromatosis type I
- Down syndrome
- Certain congenital heart defects
- Previous brain radiation therapy
- Giant craniofacial hemangiomas
Diagnostic tests for Moyamoya disease include MRI (magnetic resonance imaging) and diagnostic cerebral angiography (X-ray images of the inside of cerebral blood vessels), along with tests to assess cerebral blood flow including brain SPECT imaging, a nuclear medicine study, and quantitative MRA imaging.
Quantitative flow MRA is a diagnostic test used during the pre-treatment evaluation process. Nova® qMRA is non-invasive and produces a fully rotating 3D model to show the blood vessels from every angle. The resulting report and blood vessel images show blood flow volume, velocity and direction.
Make an appointment at our Moyamoya Center
Cushing Neuroscience Institute’s Moyamoya Center makes it easy for you to take the first steps in ensuring the best neurological care for yourself and your family. Simply click on our Request an Appointment form, email us at firstname.lastname@example.org, or call us at (516) 562-3064.