Comorbidities and predictors of health-related quality of life in Dravet syndrome: A 10-year, prospective follow-up study

Abstract

Objective: Dravet syndrome (DS) is a severe developmental and epileptic encephalopathy, leading to reduced health-related quality of life (HRQOL). Prospective outcome data on HRQOL are sparse, and this study investigated long-term predictors of HRQOL in DS. Methods: One hundred thirteen families of SCN1A-positive patients with DS, who were recruited as part of our 2010 study were contacted at 10-year follow-up, of which 68 (60%) responded. The mortality was 5.8%. Detailed clinical and demographic information was available for each patient. HRQOL was evaluated with two epilepsy-specific instruments, the Impact of Pediatric Epilepsy Scale (IPES) and the Epilepsy & Learning Disabilities Quality of Life Questionnaire (ELDQOL); a generic HRQOL instrument, the Pediatric Quality of Life Inventory (PedsQL); and a behavioral screening tool, the Strength and Difficulties Questionnaire (SDQ). Results: Twenty-eight patients were 10–15 years of age (0–5 years at baseline) and 40 were ≥16 years of age (≥6 years at baseline). Patients 0- to 5–years-old at baseline showed a significant decline in mean scores on the PedsQL total score (p =.004), physical score (p