The relationship between cerebral infarction and angiographic characteristics in childhood moyamoya disease

BACKGROUND AND PURPOSE: In childhood-onset moyamoya disease, the angiograph ic disease process of stenoocclusive lesions is progressive, and cerebral i nfarctions often develop as a result of ischemia, Our purpose was to determ ine how the severity of sternoocclusive lesions in the anterior and posteri or circulations affects the distribution of cerebral infarction in patients with childhood-onset moyamoya disease. METHODS: In 69 patients with childhood-onset moyamoya disease, angiograms w ere reviewed for stenoocclusive lesions, and CT scans, MR images, or both w ere reviewed for the sites and extent of cerebral infarction, The relations hip between the angiographic and CT/MR findings was examined. RESULTS; The prevalence and degree of stenoocclusive lesions of the posteri or cerebral artery (PCA) significantly correlated with the extent of lesion s around the terminal portion of the internal carotid artery (ICA). The pre valence of infarction significantly correlated with the degree of stenooccl usive changes of both the ICA and BCA, Infarctions tended to be distributed in the anterior borderzone in less-advanced cases, while in more advanced cases lesions were additionally found posteriorly in the territory of the m iddle cerebral artery, the posterior borderzone, and the PCA territory, CONCLUSION: Our results indicate that progressive changes of the anterior a nd posterior circulations are associated with the distribution of cerebral infarction, culminating in a patchily disseminated or honeycomb pattern of infarction on CT and MR studies in late stages of the disease.