Background: Moyamoya disease often presents white matter hyperintensity (WMH) lesions on fluid-attenuated inversion recovery (FLAIR) images, which is generally accepted as irreversible. We, herein, describe three cases of moyamoya disease with WMH lesions that regressed or disappeared after surgical revascularization.
Case description: This report included two pediatric and one young adult case that developed transient ischemic attacks or ischemic stroke due to bilateral Moyamoya disease. Before surgery, five of their six hemispheres had WMH lesions in the subcortical and/or periventricular white matter on FLAIR images. The lesions included morphologically two different patterns: "Striated" and "patchy" morphology. In all of them, combined bypass surgery was successfully performed on both sides, and no cerebrovascular events occurred during follow-up periods. On follow-up magnetic resonance examinations, the "striated" WMH lesions completely disappeared within six months, while the "patchy" WMH lesions slowly regressed over 12 months.
Conclusion: Based on radiological findings and the postoperative course of the WMH lesions, the "striated" WMH lesions may represent the inflammation or edema along the neuronal axons due to cerebral ischemia, while the "patchy" WMH lesions may represent vasogenic edema in the white matter through the blood-brain barrier breakdown. Earlier surgical revascularization may resolve these WMH lesions in Moyamoya disease.
Keywords: Cerebral ischemia; Moyamoya disease; Revascularization; White matter hyperintensity.
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