Moyamoya Disease

Moyamoya disease is a progressive cerebrovascular disorder that often begins during childhood. Patients with Moyamoya disease have injuries in their blood vessels at the base of the brain. The term ‘moyamoya’ comes from Japanese and means ‘puff of smoke’, which describes the appearance of abnormal blood vessels. Symptoms of Moyamoya disease include acute strokes, transient ischemic attacks (TIA), blurred vision, and weakness of one side of the body. Some researchers believe that Moyamoya disease is the result of inherited genetic disorders because individuals with Moyamoya often have close relatives who are also affected.

If the disease is not treated properly, patients will suffer from mental decline and recurrent strokes due to insufficient blood supply caused by moyamoya vessels in the brain. In severe cases, Moyamoya disease can cause death and life-long disabilities. The primary goal of treatment is to reduce the risk of strokes using medical and/or surgical interventions. In mild cases, medications are effective to prevent acute or recurrent strokes. In more severe cases, revascularization surgeries can restore normal blood flow bypassing affected blood vessels.

Imaging exams (such as CT or MRI) are often performed to diagnose Moyamoya disease and identify the location of the affected blood vessels. The most widely used techniques include CT perfusion, digital subtraction angiography, and MR angiography. In recent years, more advanced techniques such as arterial spin labeling have been employed to evaluate the blood flow and circulation without radiation or contrast agents.

AHA has been a pioneer in providing support for Moyamoya patients and funding research on this disease. I am honored to be supported by AHA to develop advanced imaging technologies to identify Moyamoya patients with a higher risk of stroke.

A recent story of a patient recovering from Moyamoya disease was covered by the AHA news:

Image source: Moyamoya Foundation




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