A Phase 2, Double-Blind, Randomized, Dose-Ranging Trial Of Reldesemtiv In Patients With ALS

Authors

Jeremy M. Shefner, Barrow Neurological InstituteFollow
Jinsy A. Andrews, The Neurological Institute of New York
Angela Genge, Institut-Hôpital Neurologique de Montréal
Carlayne Jackson, University of Texas Health Science Center at San Antonio
Noah Lechtzin, Johns Hopkins School of Medicine
Timothy M. Miller, Washington University School of Medicine in St. Louis
Bettina M. Cockroft, Cytokinetics, Inc.
Lisa Meng, Cytokinetics, Inc.
Jenny Wei, Cytokinetics, Inc.
Andrew A. Wolff, Cytokinetics, Inc.
Fady I. Malik, Cytokinetics, Inc.
Cynthia Bodkin, Indiana University School of Medicine
Benjamin R. Brooks, Carolinas Neuromuscular ALS MDA Clinic
James Caress, Wake Forest University Health Sciences
Annie Dionne, CHU de Québec - Université Laval
Dominic Fee, Medical College of Wisconsin
Stephen A. Goutman, University of Michigan, Ann Arbor
Namita A. Goyal, University of California, Irvine
Orla Hardiman, Beaumont Hospital, Dublin
Ghazala Hayat, St. Louis University
Terry Heiman-Patterson, Lewis Katz School of Medicine
Daragh Heitzman, Texas Neurology
Robert D. Henderson, Royal Brisbane and Women's Hospital
Wendy Johnston, University of Alberta
Chafic Karam, Oregon Health & Science University
Matthew C. Kiernan, Royal Prince Alfred Hospital
Stephen J. Kolb, The Ohio State University Wexner Medical Center
Lawrence Korngut, University of Calgary
Shafeeq Ladha, St. Joseph's Hospital and Medical CenterFollow
Genevieve Matte, Centre Hospitalier de L'Universite de Montreal
Jesus S. Mora, Hospital San Rafael

Document Type

Article

Abstract

© 2020 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group. Objective: To evaluate safety, dose response, and preliminary efficacy of reldesemtiv over 12 weeks in patients with amyotrophic lateral sclerosis (ALS). Methods: Patients (≤2 years since diagnosis) with slow upright vital capacity (SVC) of ≥60% were randomized 1:1:1:1 to reldesemtiv 150, 300, or 450 mg twice daily (bid) or placebo; active treatment was 12 weeks with 4-week follow-up. Primary endpoint was change in percent predicted SVC at 12 weeks; secondary measures included ALS Functional Rating Scale-Revised (ALSFRS-R) and muscle strength mega-score. Results: Patients (N = 458) were enrolled; 85% completed 12-week treatment. The primary analysis failed to reach statistical significance (p = 0.11); secondary endpoints showed no statistically significant effects (ALSFRS-R, p = 0.09; muscle strength mega-score, p = 0.31). Post hoc analyses pooling all active reldesemtiv-treated patients compared against placebo showed trends toward benefit in all endpoints (progression rate for SVC, ALSFRS-R, and muscle strength mega-score (nominal p values of 0.10, 0.01 and 0.20 respectively)). Reldesemtiv was well tolerated, with nausea and fatigue being the most common side effects. A dose-dependent decrease in estimated glomerular filtration rate was noted, and transaminase elevations were seen in approximately 5% of patients. Both hepatic and renal abnormalities trended toward resolution after study drug discontinuation. Conclusions: Although the primary efficacy analysis did not demonstrate statistical significance, there were trends favoring reldesemtiv for all three endpoints, with effect sizes generally regarded as clinically important. Tolerability was good; modest hepatic and renal abnormalities were reversible. The impact of reldesemtiv on patients with ALS should be assessed in a pivotal Phase 3 trial. (ClinicalTrials.gov Identifier: NCT03160898).

Publication Date

1-1-2020

Publication Title

Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

ISSN

21678421

E-ISSN

21679223

Digital Object Identifier (DOI)

10.1080/21678421.2020.1822410

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