Two adult patients with moyamoya disease presenting with alexia with agraphia limited to kanji: can revascularization improve characteristic symptoms?

Abstract

Patients with moyamoya disease (MMD) may exhibit higher brain dysfunction due to hypoperfusion, which may be ameliorated by revascularization. However, few studies have examined the relationship between cerebral perfusion and language function or the ameliorating effect of revascularization on language dysfunction. We present two cases with MMD who presented with alexia with agraphia, specifically for Japanese kanji. The patients had impaired perfusion in the left inferior temporal and lateral occipital lobes. Following superficial temporal artery-middle cerebral artery bypass, the symptoms improved dramatically. Thus, correction of hypoperfusion may be effective even in adult patients with MMD presenting with language dysfunction.