This month my literature survey returned a poster that was presented at the recent American Epilepsy Society meeting. Although this is not a peer-reviewed journal article, I include it because it is interesting, and I have no concerns about its legitimacy. In previous summaries, the story of fenfluramine has been presented; along with some results from its clinical trial and open-label extension use, which have been very positive. Given the extensive reduction in seizure frequency observed, the authors in the current work analyzed whether this correlated with improvements in executive function.

Executive function is a term that encompasses the child’s ability to regulate behavior, to regulate emotional responses and adjust to changes in the environment, and to manage cognitive processes and problem-solve effectively. It is assessed by parents and caregivers, using a standardized inventory that captures and quantifies a range of executive function components. Three index scores are produced (behavior, emotion, cognition) as well as an overall composite score.

The main finding was that a reduction in monthly convulsive seizure frequency (MCSF) was associated with an improvement in overall executive function. The correlation was most clearly seen when comparing the extremes – patients who had a profound reduction in MCSF (≥75%) were significantly more likely to show clinically meaningful improvements in overall executive function than patients who had minimal reduction in MCSF (<25%). The authors conclude that a treatment capable of producing profound reduction in MCSF, especially earlier in disease progression, may provide a great beneficial impact on executive function.

 

Bishop, KI, et al. Profound Reduction in Seizure Frequency (≥75%) Leads to Improved Everyday Executive Function: Analysis From a Phase 3 Study of ZX008 (Fenfluramine HCl) in Children/Young Adults With Dravet Syndrome. Presented at the American Epilepsy Society (AES) Annual Meeting, December 2019, Baltimore, MD.