Association Between Frontal Lobe Hemodynamics and Neurocognitive Dysfunction in Adults With Moyamoya Disease: Retrospective Cohort Analysis.

BACKGROUND: Adult patients with moyamoya disease (MMD) may present with mild cognitive dysfunction, even those without evidence of conspicuous brain parenchymal damage. This cognitive dysfunction might be caused by local frontal lobe ischemia. OBJECTIVE: To explore the relationship between frontal lobe hemodynamic insufficiency and cognitive dysfunction in patients with MMD. METHODS: Thirty adult patients with MMD without conspicuous brain parenchymal damage were retrospectively examined. Patients with MMD with frontal lobe intracerebral steal phenomenon on single photon emission computed tomography were defined as group S (n = 13) and those without it were defined as group P (n = 17). A comparative group comprising patients with unruptured intracranial aneurysm was defined as group C (n = 30). The results of various cognitive and intelligence tests and a composite cognitive score were compared between groups. RESULTS: The digit span test forward version ( P = .041), frontal assessment battery ( P = .022), and composite cognitive score ( P = .015) z-scores were significantly lower in group S than group C. Adjusting for sex and age, patients in group S had a significantly lower composite cognitive score compared with those in group C in multiple regression analysis ( P = .037). Executive dysfunction and working memory dysfunction may be involved in the cognitive decline observed in group S. CONCLUSION: Mild cognitive dysfunction in MMD was associated with frontal lobe hemodynamic insufficiency. Future studies should examine whether revascularization can improve cerebral hypoperfusion and neurocognitive function in these patients.

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

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.