Evaluation of behavioral problems in the VISION-DMD study of vamorolone vs prednisone in Duchenne muscular dystrophy


Topic:

Other

Poster Number: P106

Author(s):

Eric Henricson, PhD, MPH, University of California, Davis, Ana de Vera, MD, Santhera Pharmaceuticals (Switzerland) Ltd, Pratteln, Switzerland, Paula R Clemens, MD, University of Pittsburgh, Michela Guglieri, MD, Newcastle University, Newcastle upon Tyne, England, UK, Natalie Truba, PhD, Nationwide Children’s Hospital, Keri Kasun, PharmD, Catalyst Pharmaceuticals, Raymond Czaja, Pharm D, Catalyst Pharmaceuticals, Eric Hoffman, PhD, Reveragen BioPharma, Rockville, Maryland, United States of America

Background: Psychiatric adverse effects during systemic corticosteroid therapy are common and well documented.

Objective: To report the frequency of behavioral problems in the phase 2b VISION-DMD study (NCT03439670) using the PARS III scale, a validated index of youth psychosocial adjustment in Duchenne muscular dystrophy (DMD), that consists of 28 items in 6 subscales (peer relations, dependency, hostility, productivity, anxiety/depression and withdrawal).

Methods: Patients (N=118) were randomized to placebo (n=29), prednisone 0.75 mg/kg/day (n=31), or vamorolone at 2 (n=30) or 6 (n=28) mg/kg/day. PARS III subscales assessed by parents were normalized as z-scores using historical data. Clinically relevant worsening in PARS III subscales was defined as a shift from normal baseline adjustment score (z-score <1) to an abnormal score (z-score ≥1) at week 24. Results: Moderate or severe behavioral adverse events (BAEs) were more frequent in the prednisone group (22.6%) than in any other arm (≤3.4% in all other groups). One patient on prednisone discontinued due to a severe BAE. Vamorolone showed no moderate or severe clinically relevant BAEs. After 24 weeks, the probability of having experienced at least 1 BAE was lowest in the placebo group, followed by vamorolone 2 mg/kg/day, vamorolone 6 mg/kg/day, and prednisone. Clinical worsening in hostility was more frequent with prednisone (26.1%) than vamorolone 6 mg/kg/day (15.4%), 2 mg/kg/day (9.1%), or placebo (8.0%). Clinical worsening in dependency and productivity was reported in >20% of patients with prednisone (24.0% and 26.9%, respectively) compared with <10% in any other group. Conclusion: Vamorolone 6 mg/kg/day was associated with an increase in mainly mild BAEs compared with placebo, but with a lower frequency and severity of BAEs reported compared with prednisone. PARS III subscales showed a reduced risk for psychosocial adjustment/functioning in hostility, dependency, and productivity with vamorolone compared with prednisone.