Usefulness of preschool and school versions of the Behavioral Rating Inventory of Executive Functions in the evaluation of the daily life executive function in myoclonic-atonic epilepsy.

Purpose: Executive functions (EF) are high-order cognitive skills that have a major influence on quality of life, social skills, and school achievement. We aimed to screen EF daily life abilities in young patients with myoclonic-atonic epilepsy (MAE) using an ecological questionnaire and to correlate EF to epilepsy characteristics.

Methods: Behavioral Rating Inventory of Executive Functions - Preschool (BRIEF-P) and BRIEF - for school-aged patients - parental questionnaires were proposed to patients with MAE and typically developing children (TDC) including Inhibit, Shift, Emotional control, Working memory (WM), Plan/Organize, Initiate, Organization of materials, and Monitor subscales. We included prospectively 12 patients with MAE and 44 TDC aged 3 to 5 years and seven patients with MAE and 21 TDC aged 6-7 years. We performed in addition for all patients an intellectual efficiency evaluation using WPPSI-IV (Wechsler intelligence scale for preschool children version IV) and collected demographics, age at onset of epilepsy, epilepsy duration, response to treatment, number and type of treatments including AEDs (antiepileptic drugs), and ketogenic diet.

Results: Four out of 12 patients for BRIEF-P and 6/7 patients for BRIEF had pathological scores for at least one domain. Behavioral Rating Inventory of Executive Functions' questionnaires showed higher pathological scores for WM, Plan/Organize, Initiate, Monitor, and Metacognition Index in patients with MAE compared to TDC suggesting higher problems reported by parents. Working memory scores were higher in the group with MAE than TDC for both BRIEF-P and BRIEF. Response to treatment is a predictor of multiple BRIEF-P domains. Epilepsy duration predicts Shift and WM domains while age at onset predicts WM domain on BRIEF in this syndrome.

Conclusions: This study is the first to assess prospectively EF in young patients with MAE. We show everyday deficits in EF reported by parents. Metacognition and more specifically WM, appear to be a core deficit. Early evaluation of EF using both questionnaires and standardized tools is necessary for early detection of EF deficit and initiating tailored rehabilitation. Given the normal development before seizure onset and the absence of cerebral lesion in MAE, these results are in favor of the impact of epilepsy on EF.