Moyamoya Disease

Moyamoya disease is a rare vascular disorder characterized by progressive narrowing or occlusion of the brain's major arteries (especially the internal carotid artery and its branches). As cerebral blood flow decreases, small abnormal vessels form. These vessels appear as a "puff of smoke" on angiography; the Japanese term "moyamoya" means "cloudy smoke."

Characteristics

• In children, it presents more often with ischemic strokes (such as paralysis, transient ischemic attacks).

• In adults, it can manifest as cerebral hemorrhage (intracranial hemorrhage).

Causes

• The exact cause is unknown (idiopathic).

• In some cases, moyamoya-like syndromes can develop after conditions such as Down syndrome, neurofibromatosis type 1, or radiation therapy.

Symptoms

• Recurrent strokes or transient ischemic attacks

• Headache

• Seizures

• Cognitive decline (especially in children)

• Symptoms of bleeding (sudden loss of consciousness, severe headache)

Diagnosis

• Magnetic Resonance Angiography (MRA) or CT Angiography: These show narrowing of brain vessels and an abnormal network of small vessels.

• Catheter Angiography: The gold standard diagnostic method, clearly showing the characteristic "smoke" image.

Treatment

• Surgical revascularization: Indirect (encephaloduroarteriosynangiosis - EDAS) or direct bypass surgeries are performed to increase blood flow to the brain.

• Medical treatment: Antiplatelet medications (such as aspirin) may be used to reduce the risk of stroke.

Outcome

• If untreated, moyamoya disease can lead to serious and permanent neurological damage. Early surgical intervention can significantly improve long-term outcomes.