Moyamoya Disease

Moyamoya disease is a rare condition in which the main arteries at the base of the brain slowly narrow and close. The brain responds by growing a network of tiny, fragile vessels that looks like a puff of smoke on an angiogram, which is what the Japanese name means.

The narrowing reduces blood flow and the fragile vessels can bleed, so the disease causes strokes and mini-strokes in children and in adults, typically in their thirties and forties. Medicines do not reopen the arteries. Bypass surgery can improve blood supply and lower the risk of further strokes, but it does not repair damage already done.

Symptome

• Brief episodes of weakness, numbness or speech difficulty, often on alternating sides
• Episodes brought on by crying, blowing, hot food or exertion in children
• Established stroke
• Headaches
• Seizures
• Involuntary movements
• Decline in school performance or concentration
• Sudden severe headache from a brain bleed, more typical in adults

Ursachen und Risikofaktoren

The cause of the artery narrowing is not known. The disease is most common in East Asia, where a variant of the RNF213 gene is strongly linked to it, and about 1 in 10 patients has an affected relative. When the same artery pattern appears together with another condition, such as sickle cell disease, Down syndrome, neurofibromatosis type 1 or earlier radiotherapy to the head, it is called moyamoya syndrome. The narrowing progresses in most people, though at very different speeds.

Wie es diagnostiziert wird

• MRI and MR angiography: Show old and new strokes, the narrowed arteries and the collateral vessels.
• Catheter cerebral angiography: The definitive test. It grades the disease and maps scalp and neck arteries that could be used for a bypass.
• Perfusion imaging: SPECT, PET or perfusion MRI, sometimes with an acetazolamide challenge, shows which areas are short of blood and guides the decision to operate.
• Blood tests: To look for associated conditions such as sickle cell disease, thyroid disease or clotting disorders.

Behandlungsmöglichkeiten

• Antiplatelet medicine: Low-dose aspirin is commonly used to reduce clot-related strokes. It does not halt the narrowing.
• Direct bypass: A scalp artery is stitched to a brain surface artery (STA to MCA). It improves flow immediately and is the usual choice in adults.
• Indirect bypass: Vascularised tissue such as a scalp artery or muscle is laid on the brain so that new vessels grow in over months (EDAS, EMS and similar). Often used in children, whose arteries are very small.
• Combined bypass: Both techniques in one operation.
• Lifestyle and anaesthetic precautions: Good hydration, avoiding hyperventilation and careful blood pressure control, especially around any operation.
• Rehabilitation: Therapy after stroke for movement, speech and learning.

Wenn es dringend ist

Sudden weakness of the face, arm or leg, loss of speech, a seizure or a sudden severe headache is a stroke until proven otherwise. Call emergency services where you are. Do not wait for a planned trip, and do not fly with new symptoms.

Reisen nach Türkiye zur Behandlung

Revascularisation surgery is done in a limited number of neurosurgical units, so travelling for assessment and a planned bypass is reasonable when you are stable. Allow 2 to 3 weeks, often per side, with the second side done weeks or months later. Ask how many moyamoya bypasses the team performs a year and what their perioperative stroke rate is. Long flights and dehydration carry some risk, so discuss timing with the surgeon. Follow-up imaging at 6 to 12 months and lifelong neurology review happen at home.

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