Moyamoya Disease

Brittany, 30, was frightened when she began experiencing stroke-like symptoms while she was pregnant with her third child. After an MRI, a neurologist diagnosed her with moyamoya disease, a rare, progressive disorder caused by blocked arteries at the base of the brain.

Neurological surgeon Dr. Neil Martin explains that the cause of disease is unknown, but it results in the gradual blocking of blood flow to the brain, often leading to hemorrhagic stroke and transient or permanent brain damage. The name moyamoya, he says, is the Japanese word for “puff of smoke,” which describes the look of the tangle of vessels formed in the brain to compensate for blockages. 

According to the Mayo Clinic, moyamoya disease typically affects children and usually occurs in people from Japan and other Asian countries, though cases in North America and Europe have been reported, as well.

Common symptoms:

  • Stroke-like symptoms, including disturbed consciousness, speech deficits, sensory and cognitive impairments, involuntary movements, headaches, seizures and vision problems.
  • In children, the first symptom is typically a stroke, frequently accompanied by muscular weakness or paralysis affecting one side of the body, or seizures.
  • In adults, patients often experience a hemorrhagic stroke due to recurrent blood clots in the affected brain vessels, or progressive cognitive or learning impairment.

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Treatment options:

  • Your doctor might prescribe an aspirin regimen after diagnosis, if you have mild or no symptoms, to prevent strokes.
  • Surgical options include a direct bypass, in which surgeons attach a scalp artery directly to a brain artery to bypass the blockage and increase blood flow to the brain.

Without treatment, moyamoya disease can be fatal. Left untreated, individuals with the disease will experience mental decline and multiple strokes due to the narrowing of the arteries.

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