Definition

Moyamoya disease is a progressive narrowing of the brain’s internal carotid arteries, causing tiny collateral vessels to form, giving a “puff of smoke” appearance on angiography.

Prevalence

Rare. More common in East Asian populations but occurs worldwide.

Symptoms

• Transient ischemic attacks (TIAs)

• Stroke-like symptoms

• Headaches

• Seizures

• Cognitive decline

• Weakness, numbness, or speech problems

Diagnosis

• MRI and MRA

• Cerebral angiography (gold standard)

• CT/MRI perfusion studies

• Genetic testing in familial cases

Treatment Options

• Surgical revascularisation (direct or indirect bypass)

• Stroke prevention measures

• Symptom management

• Long-term follow-up with imaging