Evaluation and management of moyamoya disease and moyamoya syndrome

Moyamoya is generally regarded as a progressive cerebrovascular entity, but the extent and rate of progression varies among patients. Treatment for patients with impaired blood flow is surgical, but other patients with milder findings may be managed with conservative therapy and surveillance imaging.

ICA: internal carotid artery; MCA: middle cerebral artery; MMD: moyamoya disease; MMS: moyamoya syndrome; TIA: transient ischemic attack; MRA: magnetic resonance angiography; CTA: computed tomography angiography; DSA: digital subtraction angiography; TCD: transcranial Doppler; CT: computed tomography; MRI: magnetic resonance imaging; PET: positron emission tomography; SPECT: single photon emission computed tomography.

* Moyamoya vasculopathy is most frequently bilateral but may be asymmetric or initially unilateral. Vascular changes may also be found infrequently in the posterior circulation (eg, posterior cerebral arteries).

¶ MMS may be caused by several underlying conditions. Refer to the UpToDate topic for additional details on associated conditions.

Δ Evaluations for clinical or imaging progression may be performed every 12 months for asymptomatic patients. Less frequent evaluations may be appropriate for adult patients who remain stable for at least three years. Refer to the UpToDate topic for additional details.

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Evaluation and management of moyamoya disease and moyamoya syndrome