Moyamoya disease (MMD) is a rare disease that affects the blood vessels supplying the brain.
Moyamoya disease (MMD) is a rare disease affecting the blood vessels to the brain, generally on both sides. The disease is characterised by the progressive narrowing and occlusion of the intracranial internal carotid arteries and major branches due to the growth of the inner lining of the arteries. To compensate, the small branches of the vessels dilates and establish further connections inside the brain (called collateral circulation).
As the disease progresses, the dilated arteries in the base of the brain may develop small aneurysms, which can lead to rupture and brain haemorrhage.
There is a genetic predisposition to the aetiology of MMD, though the exact reason is unknown.
Diagnosis of MMD is usually one of, or a combination of a CT scan, MRI scan and a cerebral angiography to assess the anatomy of the arteries in the brain and scalp.
Patients with moyamoya disease who present for treatment while symptoms are still evolving have a better prognosis than those who present with static symptoms (which probably indicate a completed stroke). The goal of offering surgery to patients with MMD is to prevent neurologic deterioration from strokes.
Various surgical procedures have been used, and they can be divided into two groups depending on whether they involve direct or indirect anastomosis between the blood vessels. In some patients, if micro aneurysms are identified, endovascular occlusion of the vessel can be performed, particularly in association with bypass surgery.
Without surgery, the majority of individuals with moyamoya disease will experience mental decline and multiple strokes because of the progressive narrowing of arteries, and can be fatal as the result of intracerebral haemorrhage.
Surgical treatment of moyamoya disease using various surgical techniques has been shown to be safe and effective in reducing ischemic events, in both adults and children
This article is intended to inform and give insight, but not treat, diagnose or replace the advice of a doctor. Always consult a doctor for any questions regarding a medical condition.