Efficacy and tolerability of EH301 for amyotrophic lateral sclerosis: a randomized, double-blind, placebo-controlled human pilot study


Por: de la Rubia JE, Drehmer E, Platero JL, Benlloch M, Caplliure-Llopis J, Villaron-Casales C, de Bernardo N, AlarcÓn J, Fuente C, Carrera S, Sancho D, GarcÍa-Pardo P, Pascual R, JuÁrez M, Cuerda-Ballester M, Forner A, Sancho-Castillo S, Barrios C, Obrador E, Marchio P, Salvador R, Holmes HE, Dellinger RW, Guarente L and Estrela JM

Publicada: 2 ene 2019
Resumen:
Background: Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease, characterized by progressive loss of spinal and cortical motor neurons, leading to muscular atrophy, respiratory failure, and ultimately death. There is no known cure, and the clinical benefit of the two drugs approved to treat ALS remains unclear. Novel disease-modifying therapeutics that are able to modulate the disease course are desperately needed. Our objective was to evaluate the efficacy and tolerability of Elysium Health's candidate drug EH301 in people with ALS (PALS). Methods: This was a single-center, prospective, double-blind, randomized, placebo-controlled pilot study. Thirty-two PALS were recruited thanks to the collaboration of the Spanish Foundation for ALS Research (FUNDELA). Study participants were randomized to receive either EH301 or placebo and underwent evaluation for 4 months. Differences between EH301 and placebo-treated participants were evaluated based on standard clinical endpoints, including the revised ALS functional rating scale (ALSFRS-R), forced vital capacity (FVC), and the Medical Research Council (MRC) grading scale. Results: Compared to placebo, participants treated with EH301 demonstrated significant improvements in the ALSFRS-R score, pulmonary function, muscular strength, and in skeletal muscle/fat weight ratio. EH301 was shown to significantly slow the progression of ALS relative to placebo, and even showed improvements in several key outcome measures compared with baseline. Conclusions: This study provides evidence in support of the disease-modifying effects of EH301 for the treatment of ALS.

Filiaciones:
de la Rubia JE:
 a Department of Nursing , Catholic University San Vicente Mártir , Valencia , Spain

Drehmer E:
 b Department of Health and Functional Valorization , Catholic University San Vicente Martir , Valencia , Spain

Platero JL:
 a Department of Nursing , Catholic University San Vicente Mártir , Valencia , Spain

Benlloch M:
 a Department of Nursing , Catholic University San Vicente Mártir , Valencia , Spain

:
 c Primary Care Service, University Hospital La Ribera , Alcira , Spain

Villaron-Casales C:
 d Department of Physiotherapy , European University of Valencia , Valencia , Spain

de Bernardo N:
 d Department of Physiotherapy , European University of Valencia , Valencia , Spain

AlarcÓn J:
 d Department of Physiotherapy , European University of Valencia , Valencia , Spain

Fuente C:
 a Department of Nursing , Catholic University San Vicente Mártir , Valencia , Spain

Carrera S:
 e Department of Health Sciences , Catholic University San Vicente Martir , Valencia , Spain

Sancho D:
 a Department of Nursing , Catholic University San Vicente Mártir , Valencia , Spain

GarcÍa-Pardo P:
 f Department of Medicine , University Jaume I , Castellón , Spain

Pascual R:
 g Rehabilitation Service, General University Hospital , Valencia , Spain

JuÁrez M:
 h Clínica Artes & Timefit , Valencia , Spain

Cuerda-Ballester M:
 a Department of Nursing , Catholic University San Vicente Mártir , Valencia , Spain

Forner A:
 a Department of Nursing , Catholic University San Vicente Mártir , Valencia , Spain

Sancho-Castillo S:
 a Department of Nursing , Catholic University San Vicente Mártir , Valencia , Spain

Barrios C:
 i Institute for Research on Musculoskeletal Disorders , Catholic University San Vicente Mártir , Valencia , Spain

Obrador E:
 j Department of Physiology , University of Valencia , Valencia , Spain

Marchio P:
 k Virgen del Consuelo Hospital , Valencia , Spain

Salvador R:
 j Department of Physiology , University of Valencia , Valencia , Spain

Holmes HE:
 l Elysium Health, Inc. , New York , NY , USA and

Dellinger RW:
 l Elysium Health, Inc. , New York , NY , USA and

Guarente L:
 l Elysium Health, Inc. , New York , NY , USA and

 m Department of Biology and Glenn Laboratories for the Science of Aging , MIT , Cambridge , MA , USA

Estrela JM:
 j Department of Physiology , University of Valencia , Valencia , Spain
ISSN: 21679223





Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Editorial
Informa Healthcare, 2-4 PARK SQUARE, MILTON PARK, ABINGDON OR14 4RN, OXON, ENGLAND, Reino Unido
Tipo de documento: Article
Volumen: 20 Número: 1-2
Páginas: 115-122
WOS Id: 000467740300015
ID de PubMed: 30668199
imagen Green Published

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