Neurocognitive profile of pediatric moyamoya disease with posterior cerebral artery involvement

While posterior cerebral artery (PCA) involvement in moyamoya disease (MMD) is associated with poor educational and social outcomes, its specific impact on neurocognitive function remains unclear. The aim of this study was to clarify the neurocognitive profile of pediatric patients with MMD with PCA involvement.

This cross-sectional study included pediatric patients with MMD who underwent baseline neuropsychological assessment at a single institution between June 2016 and December 2023. Neurocognitive function was evaluated using the Das Naglieri Cognitive Assessment (CAS) system and the Wechsler Intelligence Scale for Children, Fourth Edition (WISC-IV). Profiles were compared between patients with and without PCA involvement using multivariate ANOVA (MANOVA), followed by multiple linear regression analyses. Regional cerebral blood flow was assessed using 3D stereotactic surface projection analysis and the stereotactic extraction estimation method.

Fifty-two patients, including 11 with PCA involvement and 41 without, were analyzed. MANOVA revealed significant overall between-group differences across the 4 CAS domains and the 4 WISC-IV index scores. Post hoc comparisons showed that patients with PCA involvement had significantly lower scores in the CAS planning domain (p < 0.001) and WISC-IV processing speed index (p = 0.002), both of which involve timed tasks requiring visual scanning, number matching, connecting ordered sequences, and symbol coding. PCA involvement remained significantly associated with these deficits after adjustment for confounders. Patients with PCA involvement exhibited more severe hypoperfusion in the temporo-parieto-occipital regions than those without PCA involvement.

Patients with MMD with PCA involvement exhibited a distinct neurocognitive profile characterized by weakness in visual processing speed and visual search, accompanied by hypoperfusion in the temporo-parieto-occipital regions. Early identification of these deficits could facilitate targeted educational and rehabilitative support, and long-term postoperative evaluation is required.