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Moyamoya Disease

Progressive cerebrovascular disorder marked by chronic stenosis of the distal internal carotid arteries with development of fragile collateral 'puff of smoke' vessels at the base of the brain, predisposing to ischemic stroke in children and hemorrhagic stroke in adults.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Nöroloji department. Book Appointment →

What is Moyamoya Disease?

Moyamoya disease is an idiopathic chronic progressive narrowing of the terminal internal carotid arteries and proximal anterior and middle cerebral arteries, leading to formation of fine collateral vessels at the base of the brain — the 'moyamoya' (puff of smoke) on angiography.

It is bilateral by definition, with peaks in childhood (5 to 10 years) presenting with transient ischemic attacks or strokes, and in adulthood (30 to 50 years) presenting with intracerebral or subarachnoid hemorrhage.

Moyamoya syndrome refers to a similar angiographic appearance secondary to neurofibromatosis, sickle cell disease, prior radiation, Down syndrome, or other vasculopathies.

Symptoms

Recurrent transient ischemic attacks or ischemic stroke in children
Headache, seizure, choreiform movements, or cognitive decline
Hemorrhagic stroke with sudden focal deficit, severe headache, or coma in adults
Hyperventilation-induced ischemia and 'rebuild-up' phenomenon on EEG in children
Progressive neurologic decline despite medical therapy

Risk Factors

Asian ancestry, particularly Japanese and Korean
Family history of moyamoya disease (RNF213 gene variant)
Sickle cell disease, neurofibromatosis type 1, Down syndrome
Prior cranial radiation in childhood
Female sex with bimodal age distribution

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Recurrent stroke-like episodes in a child or young adult
  • New focal deficit with stenosis of internal carotid artery on imaging
  • Cognitive decline or seizures with characteristic angiographic pattern
  • Hemorrhagic stroke without classical risk factors

Treatment Methods

01
Antiplatelet therapy to reduce thromboembolic risk in ischemic phenotype
02
Avoidance of hypovolemia, hypotension, and hyperventilation that worsen ischemia
03
Direct surgical revascularization with superficial temporal artery to middle cerebral artery (STA-MCA) bypass
04
Indirect revascularization with encephalo-duro-arterio-synangiosis (EDAS) or pial synangiosis in children
05
Long-term neurology follow-up with MRI, MRA, and perfusion imaging to monitor progression and graft patency

Which Department to Visit?

You can visit our Nöroloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.