Cerebrovascular Collaterals Correlate with Disease Severity in Adult North American Patients with Moyamoya Disease

BACKGROUND AND PURPOSE: Cerebrovascular collaterals have been increasingly recognized as predictive of clinical outcomes in Moyamoya disease in Asia. The aim of this study was to characterize collaterals in North American adult patients with Moyamoya disease and to assess whether similar correlations are valid.

MATERIALS AND METHODS: Patients with Moyamoya disease (n = 39; mean age, 43.5 ±10.6 years) and age- and sex-matched control subjects (n = 33; mean age, 44.3 ± 12.0 years) were graded via angiography. Clinical symptoms of stroke or hemorrhage were graded separately by imaging. Correlations between collateralization and disease severity, measured by the modified Suzuki score, were evaluated in patients with Moyamoya disease by fitting a regression model with clustered ordinal multinomial responses.

RESULTS: The presence of leptomeningeal collaterals (P = .008), dilation of the anterior choroidal artery (P = .01), and the posterior communicating artery/ICA ratio (P = .004) all correlated significantly with disease severity. The presence of infarct or hemorrhage and posterior steno-occlusive disease did not correlate significantly with the modified Suzuki score (P = .1). Anterior choroidal artery changes were not specific for hemorrhage. Patients with Moyamoya disease were statistically more likely than controls to have higher posterior communicating artery/ICA ratios and a greater incidence of leptomeningeal collaterals.

CONCLUSIONS: As with Moyamoya disease in Asian patients, the presence of cerebrovascular collaterals correlated with the modified Suzuki score for disease severity in North American patients with Moyamoya disease. However, anterior choroidal artery changes, which correlated with increased rates of hemorrhage in Asian studies, were not specific to hemorrhage in North Americans.