Ewha Med J.  2013 Mar;36(1):9-17. 10.12771/emj.2013.36.1.9.

Diagnosis and Treatment of Moyamoya Disease

• Seo EK

• KMID: 2284010
• DOI: http://doi.org/10.12771/emj.2013.36.1.9

Abstract

Moyamoya disease is a cerebrovascular disease of unknown etiology, which is characterized by bilateral stenosis or occlusion at terminal portion of internal carotid artery and at proximal portion of anterior cerebral artery and/or middle cerebral artery and abnormal vascular network in the vicinity of the arterial occlusions. It occurs frequently in Asian countries, particularly in Korea and Japan, but is rare in Western countries. To establish the etiology of moyamoya disease, much about the pathology from autopsies, factors involved in its pathogenesis, and its genetics have been studied. It may occur at any age from childhood to adulthood and in general, initial manifestation is cerebral ischemic symptoms in children and intracranial hemorrhage symptoms in adults. Because it progress and cause recurrent stroke, early diagnosis and proper management has been recognized. Cerebral angiography is essential for definitive diagnosis and treatment plan. Magnetic resonance imaging/magnetic resonance angiography is useful for diagnosis and follow-up tools after revascularization. Evaluation of the cerebral hemodynamics by single photon emission computed tomography and positron emission tomography is useful for diagnosis and assessment of the severity of cerebral ischemia in moyamoya patients. Surgical revascularization is effective for moyamoya disease manifesting as ischemic symptoms, to prevent further ischemia and infarction. In hemorrhagic type moyamoya disease, revascularization can be considered. Direct bypass, indirect synangiosis and combined methods are used. Outcomes of revascularization are excellent in preventing transient ischemic attacks in most patients.

Keyword

Cerebral hemorrhage; Cerebral ischemia; Moyamoya disease; Revascularization

MeSH Terms

Adult
Angiography
Anterior Cerebral Artery
Asian Continental Ancestry Group
Autopsy
Brain Ischemia
Carotid Artery, Internal
Cerebral Angiography
Cerebral Hemorrhage
Child
Constriction, Pathologic
Early Diagnosis
Follow-Up Studies
Hemodynamics
Humans
Infarction
Intracranial Hemorrhages
Ischemia
Ischemic Attack, Transient
Japan
Korea
Magnetic Resonance Spectroscopy
Middle Cerebral Artery
Moyamoya Disease
Positron-Emission Tomography
Stroke
Tomography, Emission-Computed, Single-Photon

Figure

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  Fig. 1 A left carotid angiography (A, anteroposterior view; B, lateral view) shows severe stenosis in terminal portion of internal carotid artery and proximal middle cerebral artery and occlusion of proximal portion of anterior cerebral artery and demonstrates basal collateral vessels. A right carotid angiography (C, anteroposterior view; D, lateral view) demonstrates stenosis of proximal middle cerebral artery and occlusion in proximal portion of anterior cerebral artery.

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  Fig. 2 Initial angiography (A) shows mild stenosis in terminal portion of internal carotid artery and well visualized middle cerebral artery. After 2 years, angiography (B) demonstrate that middle cerebral artery is not visualized and basal there are collateral vessels (arrow head) from posterior cerebral artery. Posterior cerebral artery is occluded and collateral vessel is disappeared after 6 months later (C).

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  Fig. 3 A T2 weighted axial magnetic resonance image (A) shows cerebral infarction in right temporo-occipital areas. An axial T1 weighted image (B) demonstrates multiple signal voids of the hypertrophied moyamoya collateral in the basal ganglia.

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  Fig. 4 Basal single photon emission computed tomography (SPECT) (A) and acetazolamide SPECT (B) show decreased blood flow and reserve capacity in left frontal lobe.

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  Fig. 5 After revascularization, cerebral blood flow is increased in left frontal lobe.

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