Aim: To understand the wide variety of clinical outcomes in children with agenesis of the corpus callosum (AgCC) and examine evidence for the proposed neuropsychological syndrome reported in adults with primary AgCC.
Method: PsycINFO, PsycArticles, Medline, Embase, and Web of Science (January 2007-November 2021) were searched to identify studies reporting on cognitive or neuropsychological outcome in children with AgCC aged up to 18 years. Twenty-three articles investigating the cognitive profile were found; their methodology was evaluated against quality criteria.
Results: While there was a high degree of heterogeneity across studies, including the methodological quality, there was evidence for some features of the neuropsychological syndrome in children with AgCC. Vulnerabilities in executive function and social cognition were found, with particular difficulties on complex and novel tasks.
Interpretation: Data on the neuropsychological outcomes in children with AgCC are limited. Broad assessments are necessary to determine the extent to which core features of the neuropsychological syndrome may characterize children with AgCC and how additional neuroanatomical features contribute to outcome.
© 2023 The Authors. Developmental Medicine & Child Neurology published by John Wiley & Sons Ltd on behalf of Mac Keith Press.