Preoperative neurodevelopment of children with moyamoya angiopathy.

AIM: To preoperatively assess the neurodevelopment of a predominantly white population of children with moyamoya angiopathy (MMA).

METHOD: Assessments of 40 children with MMA (24 females, 16 males; mean age 6y 11mo, range 20mo-16y) included tests for non-verbal IQ and fine motor skills, and questionnaires on quality of life, behaviour, and executive functions. The Paediatric Stroke Outcome Measure (PSOM) score was evaluated by a paediatric neurologist.

RESULTS: Children with MMA had significantly lower non-verbal IQ scores (mean IQ 92.1, SD 19.6, p=0.015) and fine motor skills (z-score -1.84, p=0.004) than population norms. Patients with posterior cerebral artery (PCA) involvement had poorer non-verbal IQ scores than those without (79.6, SD 24.6 vs 95.2, SD 17.2, p=0.042). Higher PSOM scores were related to lower non-verbal IQ scores (Spearman's rank correlation coefficient -0.43, p=0.006), while the presence of stroke, bilaterality, disease versus syndrome, and age at diagnosis had no significant effect on non-verbal IQ. Quality of life, behaviour, and executive functions were in the typically developing range.

INTERPRETATION: Children with MMA are more likely to manifest intellectual and fine motor skill impairment before surgical intervention. PCA involvement is an additional risk factor for lower non-verbal IQ.

WHAT THIS PAPER ADDS: Children with moyamoya angiopathy have intellectual and fine motor skill impairment before surgical intervention. Posterior cerebral artery involvement and higher Paediatric Stroke Outcome Measure scores may predict poorer performance.