What is Moyamoya Disease?

Moyamoya disease is a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called the basal ganglia. The name “moyamoya” means “puff of smoke” in Japanese and describes the look of the tangle of tiny vessels formed to compensate for the blockage.  Moyamoya disease was first described in Japan in the 1960s and it has since been found in individuals in the  United States, Europe,  Australia, and Africa.

What Causes Moyamoya Disease?

Because it tends to run in families, researchers think that Moyamoya disease is the result of inherited genetic abnormalities.  Studies that look for the abnormal gene(s) may help reveal the biomechanisms that cause the disorder.

Symptoms of Moyamoya

The disease primarily affects children, but it can also occur in adults.   In children, the first symptom of Moyamoya disease is often stroke, or recurrent transient ischemic attacks (TIA, commonly referred to as “mini-strokes”), frequently accompanied by muscular weakness or paralysis affecting one side of the body, or seizures.

Adults most often experience a hemorrhagic stroke due to recurring blood clots in the affected brain vessels. Individuals with this disorder may have

  • Disturbed consciousness
  • Speech deficits (usually aphasia)
  • Sensory and cognitive impairments
  • Involuntary movements
  • Vision problems

Treatment for Moyamoya Disease

Treating Moyamoya disease involves managing symptoms, improving blood flow to the brain, and controlling seizures. Your treatment will be uniquely related to your specific condition and adjusted accordingly.

In more advanced cases, revascularization can help patients. This surgical procedure aims to rebuild the blood supply to the underside of the brain, a delicate procedure that should be performed by a highly experienced vascular neurosurgeon.

Moyamoya Treatment at the University of Illinois Neurosurgery Department

Our team of neurosurgeons specializes in the treatment and diagnosis of rare and complex neurological conditions and disorders. We often treat patients who have been unable to receive the care they truly need. If you are suffering from a complex neurological condition, refer to our contact page to correspond with a member of our team about a consultation!