MoyaMoya

What Is Moyamoya Disease?

Moyamoya disease is a rare, progressive cerebrovascular disorder caused by narrowing or blockage of the internal carotid arteries at the base of the brain. To compensate, the brain forms tiny, fragile blood vessels that resemble a “puff of smoke” on imaging—hence the name Moyamoya, which is Japanese for “hazy, like smoke.”

Who It Affects

• Most common in children aged 5–10 and adults aged 30–50
• More prevalent in East Asian populations, especially Japan, Korea, and China
• Can be genetic—some cases run in families

Symptoms of Moyamoya Disease

Symptoms result from reduced blood flow or bleeding in the brain:

• Transient ischemic attacks (TIAs) or “mini-strokes”
• Hemorrhagic strokes (bleeding in the brain)
• Seizures
• Headaches
• Weakness or paralysis on one side of the body
• Vision problems
• Speech difficulties
• Cognitive or developmental delays
• Involuntary movements

Causes and Risk Factors

While the exact cause is unknown, contributing factors include:

• Genetic mutations (e.g., RNF213 gene)
• Associated conditions: Down syndrome, sickle cell disease, neurofibromatosis type 1, prior radiation therapy
• Autoimmune or connective tissue disorders
• Female gender and Asian ethnicity are higher risk

Diagnosis

Diagnosis typically involves:

• MRI/MRA or CT/CTA scans
• Cerebral angiography (gold standard)
• EEG (especially in children)
• SPECT or PET scans to assess blood flow

Treatment Options

1. Medical Management

• Antiplatelet medications (e.g., aspirin) to reduce stroke risk
• Seizure control medications
• Monitoring and lifestyle adjustments

Surgical Revascularization

• Direct bypass: connects scalp artery to brain artery
• Indirect bypass: uses muscle or tissue to promote new vessel growth (e.g., EDAS, EMS)
• Omental transposition: abdominal tissue laid on brain surface to stimulate blood flow

Ready to Take the Next Step?

Call us at (561) 433-4444 to schedule a consultation or make an appointment.
Let our experienced doctors help you manage Moyamoya disease and improve your quality of life.