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Moyamoya – Causes, Symptoms and Treatment

Moyamoya is a rare progressive cerebrovascular disease in which the major brain arteries narrow, increasing the risk of stroke in both children and adults.

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Moyamoya is a rare progressive cerebrovascular disease in which the major brain arteries narrow, increasing the risk of stroke in both children and adults.

What is Moyamoya?

Moyamoya is a rare, progressive disease of the blood vessels supplying the brain. The name comes from Japanese and translates roughly to “a puff of smoke” – describing the hazy appearance of abnormal compensatory vessels visible on angiography. In this condition, the internal carotid arteries and the large arteries at the base of the brain gradually narrow or become completely blocked. In response, the body develops a network of tiny collateral vessels that attempt to compensate for the reduced blood flow but are often insufficient.

Causes

The exact cause of Moyamoya disease is not yet fully understood. Two forms are distinguished:

Idiopathic Moyamoya disease: No identifiable underlying condition; genetic factors are involved. Mutations in the RNF213 gene have been frequently identified in affected individuals.
Moyamoya syndrome: Occurs secondary to another condition, such as sickle cell disease, Down syndrome, neurofibromatosis type 1, prior cranial radiation, or certain autoimmune disorders.

The disease is more prevalent in East Asia but affects people worldwide. There are two peak age groups: children between 5 and 10 years of age, and adults between 30 and 50 years of age.

Symptoms

Symptoms depend on whether the reduced blood flow leads to an ischemic stroke (insufficient blood supply) or a brain hemorrhage (bleeding).

Common symptoms in children

Recurrent strokes or transient ischemic attacks (TIAs)
Weakness or paralysis on one side of the body
Speech difficulties
Headaches, especially triggered by crying or hyperventilation
Seizures
Cognitive impairment and developmental delays

Common symptoms in adults

Intracranial hemorrhage (hemorrhagic stroke)
Severe headaches
Visual disturbances
Altered consciousness

Diagnosis

Diagnosis is established through neuroimaging techniques:

MRI (Magnetic Resonance Imaging): Detects areas of ischemia or hemorrhage and reveals the typical “puff of smoke” appearance of dilated collateral vessels.
MR angiography or CT angiography: Visualizes the narrowed arteries and the collateral vessel network.
Digital subtraction angiography (DSA): Considered the gold standard for diagnosis, providing high-resolution images of the vascular changes.
Perfusion imaging: Assesses cerebral blood flow and identifies areas of reduced perfusion.

Treatment

There is currently no medication that can halt the progression of vascular changes in Moyamoya. Treatment focuses on improving blood supply to the brain.

Surgical revascularization

The primary treatment approach is surgical restoration of cerebral blood flow (revascularization). Three main strategies are used:

Direct bypass surgery (STA-MCA bypass): The superficial temporal artery from the scalp is directly connected to a brain artery (middle cerebral artery) to restore blood flow.
Indirect revascularization: Well-vascularized tissue (such as the temporalis muscle or the dura mater) is placed on the brain surface to encourage new vessel growth (techniques include EDAS, EMS, and EDAMS).
Combined procedures: A combination of direct and indirect techniques for broader coverage.

Medical management

Antiplatelet therapy (e.g., aspirin) to reduce the risk of blood clots in ischemic presentations
Management of cardiovascular risk factors such as hypertension or diabetes

Rehabilitation

Following a stroke, early and intensive rehabilitation is essential to maximize functional recovery. This includes physical therapy, occupational therapy, and speech-language therapy tailored to the individual needs of the patient.

Prognosis

The prognosis depends strongly on how early the disease is diagnosed and treated. Without surgical intervention, the condition often progresses, leading to repeated strokes and increasing neurological disability. After successful revascularization, the risk of further strokes can be significantly reduced. Children in particular tend to show favorable long-term outcomes following surgery.

References

Kuroda S, Houkin K. Moyamoya disease: current concepts and future perspectives. The Lancet Neurology. 2008;7(11):1056-1066.
Scott RM, Smith ER. Moyamoya disease and moyamoya syndrome. New England Journal of Medicine. 2009;360(12):1226-1237.
Fung LW et al. Pediatric moyamoya disease: an evidence-based practice update. Seminars in Pediatric Neurology. 2012;19(3):84-96.

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