Conditions We Treat
Blood Vessels
Moyamoya Disease
Moyamoya disease is a rare and progressive condition that affects the blood vessels in the brain. It occurs when the arteries at the base of the brain (particularly the internal carotid arteries) become narrowed or blocked, leading to reduced blood flow. The body tries to compensate by creating a network of tiny blood vessels to bypass the blockage, which appears like a “puff of smoke” on imaging scans—hence the name, “moyamoya,” which means “hazy” or “puff of smoke” in Japanese.
Symptoms of Moyamoya disease can vary depending on the severity of the condition and age of the patient. Common symptoms include:
- Stroke or transient ischemic attacks (TIAs): Sudden weakness, numbness, or difficulty speaking
- Seizures: Common in children with Moyamoya disease
- Headaches: Persistent or severe headaches due to reduced blood flow
- Cognitive and developmental delays: In children, this can manifest as difficulty in learning or concentrating
- Vision problems: Blurred or double vision
- Paralysis or weakness: On one side of the body (hemiparesis)
What are the symptoms?
What are the causes and risk factors?
The exact cause of Moyamoya disease is unknown, but several factors and associations have been identified:
Genetics
Moyamoya disease is sometimes hereditary and linked to certain genetic mutations.
Age
Most commonly affects children under 10, but it can also occur in adults, particularly between 30 and 50 years old.
Ethnicity
More prevalent in East Asian populations, particularly in Japan, Korea, and China.
Associated conditions
It may occur alongside other conditions like Down syndrome, neurofibromatosis type 1, and sickle cell anaemia.
What are the complications from the condition?
- Recurrent strokes or TIAs: Due to poor blood flow to the brain
- Haemorrhagic strokes: Caused by bleeding from the fragile network of blood vessels
- Cognitive decline: Ongoing lack of oxygen can impair brain function
- Seizures: Frequent in children with advanced disease
- Death: Severe cases can lead to life-threatening strokes
How is it diagnosed?
Diagnosis involves a combination of imaging tests to confirm the presence of narrowed arteries and the characteristic “puff of smoke” appearance:
- MRI or CT Scan: Used to identify strokes, bleeding, or narrowed vessels
- Cerebral Angiography: The gold standard for diagnosing Moyamoya disease; uses dye and X-rays to visualize blood vessels.
- MRA or CTA: Non-invasive techniques to examine blood vessel abnormalities
- Electroencephalogram (EEG): May detect abnormal brain activity, especially in children with seizures
How is it treated?
Treatment aims to improve blood flow to the brain, reduce symptoms, and prevent strokes. Options include:
- Surgical Interventions: The most effective treatment involves improving blood flow using bypass surgery. Types include:
- Direct Bypass: Connecting a healthy artery outside the skull to an artery inside the brain.
- Indirect Bypass: Encouraging new blood vessel growth by redirecting tissue to the brain's surface.
- Medications: Used to manage symptoms or reduce stroke risk, such as antiplatelet drugs (e.g., aspirin) or blood pressure medications
- Therapies: Physical and occupational therapy for stroke recovery
- Monitoring: In mild cases, doctors may closely observe the condition with regular check-ups.
Frequently Asked Questions
Is Moyamoya disease curable?
Moyamoya disease cannot be cured, but treatments like surgery can significantly reduce symptoms and risks.
Can Moyamoya disease come back after treatment?
The disease can progress in untreated areas, so regular follow-ups are essential.
Is Moyamoya disease fatal?
Without treatment, severe strokes or bleeding can be fatal. However, with proper treatment, most patients can live normal lives.
Can it be prevented?
Moyamoya disease cannot be prevented, but early detection and treatment can prevent complications.
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