LAUSR

Abstract Objective: Agenesis of the corpus callosum (AgCC) is associated with a range of cognitive deficits, including mild to moderate problems in higher order executive functions evident in neuropsychological assessments. Previous research has also suggested a lack of self-awareness in persons with AgCC. Method: We investigated daily executive functioning and self-awareness in 36 individuals with AgCC by analyzing self-ratings on the Behavior Rating Inventory of Executive Function-Adult Version (BRIEF-A), as well as ratings on the same instrument from close relatives. Discrepancies between self- and informant-ratings were compared to the normative sample and exploratory analyses examined possible moderating effects of participant and informant characteristics. Results: Significant deficiencies were found in the Behavioral Regulation and Metacognitive indices for both the self and informant results, with elevated frequency of metacognition scores in the borderline to clinical range. Informants also endorsed elevated frequency of borderline to clinically significant behavioral regulation scores. The proportion of AgCC participants whose self-ratings indicated less metacognitive impairment than informant-ratings was greater than in the normative sample. Self-ratings of behavioral regulation impairment decreased with age and informant-ratings of metacognition were higher in males than females. Conclusions: These findings provide evidence that individuals with AgCC experience mild to moderate executive functioning problems in everyday behavior which are observed by others. Results also suggest a lack of self-understanding or insight into the severity of these problems in the individuals with AgCC, particularly with respect to their metacognitive functioning.