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Record W4408778571 · doi:10.1001/jamaneurol.2025.0241

Reldesemtiv in Amyotrophic Lateral Sclerosis

2025· article· en· W4408778571 on OpenAlexaff
Jeremy M. Shefner, Merit Cudkowicz, Angela Genge, Orla Hardiman, Ammar Al‐Chalabi, Jinsy Andrews, Adriano Chiò, Philippe Corcia, Philippe Couratier, Mamede de Carvalho, Terry Heiman‐Patterson, Robert D. Henderson, Caroline Ingre, Wendy Johnston, Albert C. Ludolph, Nicholas J. Maragakis, Jonathan Mill, Jesús S. Mora, Susanne Petri, Zachary Simmons, Leonard H. van den Berg, Lorne Zinman, Stuart Kupfer, Fady I. Malik, Lisa Meng, Tyrell Simkins, Jenny Wei, Andrew A. Wolff, Stacy A. Rudnicki, Matthew C. Kiernan, David Schultz, Merrilee Needham, Steve Vucic, Geneviève Matte, Colleen O’Connell, Lawrence Korngut, Annie Dionne, John Turnbull, Kerri Schellenberg, Christen Shoesmith, Jocelyn Zwicker, Lisette Salveson, Emilien Bernard, F. Salachas, Véronique Danel, Shahram Attarian, Marie‐Hélène Soriani, Annekathrin Rödiger, Julian Großkreutz, Patrick Weydt, Thomas Meyer, Vincenzo Silani, F. Cerri, Andrea Calvo, Christian Lunetta, Magdelena Kusma-Kozakiewicz, Mónica Povedano, Christer Nilsson, Markus Weber, Caroline Young, Deborah Bradshaw, Sabrina Paganoni, Jeffrey Statland, James Grogan, Jeffrey Rothstein, Bjorn Oskarrson, Thanh‐Huyen T. Vu, Nicholas Olney, Lewis Ra, Dianna Quan, Leonard H. Mccluskey, Namita Goyal, Pantellis Pavlakis, Andrea Swenson, Elham Bayat, Stephen A. Goutman, Dominic B. Fee, Peter D. Creigh, Amanda Peltier, Daragh Heitzman, Gary Patee, Cynthia Bodkin, Ximena Arcilla-London, Nizar Chahin, Shafeeq Ladha, Yuen T. So, Kourosh Rezania, Rup Tandan, Rebecca M. Kuenzler, Kelly Gwathmey, Michael Pulley

Bibliographic record

VenueJAMA Neurology · 2025
Typearticle
Languageen
FieldMedicine
TopicAmyotrophic Lateral Sclerosis Research
Canadian institutionsHealth Sciences CentreSunnybrook Health Science CentreUniversity of AlbertaMontreal Neurological Institute and Hospital
FundersNational Center for Advancing Translational Sciences
KeywordsAmyotrophic lateral sclerosisMedicinePlaceboInterim analysisRiluzolePhysical therapyClinical endpointClinical trialAdverse effectRandomized controlled trialRating scaleInternal medicineDiseasePsychology

Abstract

fetched live from OpenAlex

Importance: Treatment options for amyotrophic lateral sclerosis (ALS) remain suboptimal. Results from a phase 2 study of reldesemtiv in ALS suggested that it may slow disease progression. Objective: To assess the effect of reldesemtiv vs placebo on functional outcomes in ALS. Design, Setting, and Participants: A Study to Evaluate the Efficacy and Safety of Reldesemtiv in Patients With Amyotrophic Lateral Sclerosis (COURAGE-ALS) was a double-blind, placebo-controlled phase 3 randomized clinical trial conducted at 83 ALS centers in 16 countries from August 2021 to July 2023. The first 24-week period was placebo controlled vs reldesemtiv. All participants received reldesemtiv during the second 24-week period with a 4-week follow-up. Two interim analyses were planned, the first for futility and the second for futility and possible resizing. This was a hybrid decentralized trial with approximately half the trial visits performed remotely and the remaining visits in the clinic. Eligible participants met criteria for definite, probable, or possible ALS with lower motor neuron signs by modified El Escorial Criteria, ALS symptoms for 24 months or less, ALS Functional Rating Scale-Revised (ALSFRS-R) total score of 44 or less, and forced vital capacity of greater than or equal to 65% of predicted. Interventions: Oral reldesemtiv, 300 mg, or placebo twice daily. Main Outcomes and Measures: The primary end point was change in ALSFRS-R total score from baseline to week 24. Results: Of the 696 participants screened, 207 were screen failures. A total of 486 participants (mean [SD] age, 59.4 [10.9] years; 309 male [63.6%]) were randomized to reldesemtiv (n = 325) or placebo (n = 161); 3 randomized patients were not dosed. The second interim analysis at 24 weeks after randomization included 256 participants. The data monitoring committee recommended that the trial should end due to futility, and the sponsor agreed. The mean (SE) group difference in the ALSFRS-R score from baseline to week 24 was -1.1 (0.53; 95% CI, -2.17 to -0.08; P = .04, favoring placebo). Given excess missing data from early termination, the combined assessment assumed greater importance; it, too, failed to show a benefit from treatment with reldesemtiv (win probability was 0.44 for reldesemtiv and 0.49 for placebo, with a win ratio of 0.91; 95% CI of win ratio, 0.77-1.10; P = .11). Conclusions and Relevance: This randomized clinical trial failed to demonstrate efficacy for reldesemtiv in slowing functional decline in ALS. Trial Registration: ClinicalTrials.gov Identifier: NCT04944784.

Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.

How this classification was reachedexpand

Full frame distilled prediction

Teacher imitation

Not calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.

metaresearch head score (Codex)0.000
metaresearch head score (Gemma)0.000
Version: codex-gemma-dda1882f352aValidation status: machine_predicted_unvalidated
Candidate categoriesnone
Consensus categoriesnone
DomainCandidate signal: none · Consensus signal: none
Study designCandidate signal: Observational · Consensus signal: Observational
GenreCandidate signal: Empirical · Consensus signal: Empirical
Teacher disagreement score0.242
Threshold uncertainty score0.575

Codex and Gemma teacher scores by category

CategoryCodexGemma
Metaresearch0.0000.000
Meta-epidemiology (narrow)0.0000.000
Meta-epidemiology (broad)0.0000.000
Bibliometrics0.0000.001
Science and technology studies0.0000.000
Scholarly communication0.0000.000
Open science0.0000.000
Research integrity0.0000.001
Insufficient payload (model declined to judge)0.0000.000

Machine scores (provisional)

The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.

Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.

Opus teacher head0.027
GPT teacher head0.299
Teacher spread0.272 · how far apart the two teachers sit on this one work
Validation statusscore_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it

Classification

machine, unvalidated

Machine predicted; a candidate call from one teacher head, not a consensus.

The models applied no category: nothing in the taxonomy fit this work.
Study designObservational
Domainnot available
GenreEmpirical

How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".

Quick stats

Citations5
Published2025
Admission routes1
Has abstractyes

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