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A Study to Evaluate Efficacy, Safety and Tolerability of CK-2127107 in Patients With Amyotrophic Lateral Sclerosis (ALS)

Phase 2
Completed
Conditions
Amyotrophic Lateral Sclerosis
Interventions
Drug: Placebo
Registration Number
NCT03160898
Lead Sponsor
Cytokinetics
Brief Summary

The purpose of this study was to assess the effect of CK-2127107 (hereafter referred to as reldesemtiv) versus placebo on respiratory function and other measures of skeletal muscle function in patients with ALS.

Detailed Description

This was a double-blind, randomized, placebo-controlled, dose ranging study of reldesemtiv in patients with ALS. Eligible patients were randomized (1:1:1:1) to receive placebo or one of three doses of reldesemtiv (150, 300, or 450 mg twice daily) for 12 weeks. Randomization was stratified by riluzole concomitant use/non-use and edaravone concomitant use/non-use. Concomitant riluzole and edaravone were allowed as long as the riluzole dose had been stable for at least 30 days prior to screening and edaravone had been taken for 2 cycles prior to screening; these drugs could not be initiated during the study.

A total of 7 study visits were planned: screening, Day 1 (first dosing day), Weeks 2, 4, 8, and 12, and follow-up (4 weeks after the last dose of study drug). Study drug (placebo or reldesemtiv) was to be taken twice daily, approximately 12 hours (± 2 hours) apart and within 2 hours following a meal.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
458
Inclusion Criteria
  • Diagnosis of familial or sporadic ALS ≤ 24 months prior to screening
  • Upright Slow Vital Capacity (SVC) ≥ 60% of predicted for age, height and sex at screening
  • Able to swallow tablets
  • A caregiver (if one is needed)
  • Able to perform reproducible pulmonary function tests
  • Pre-study clinical laboratory findings within the normal range or, if outside the normal range, deemed not clinically significant by the Investigator
  • Male patients who have not had a vasectomy and confirmed zero sperm count must agree after receiving the first dose of study drug until 10 weeks after the last dose to either use acceptable methods of contraception or abstain from sex
  • Female patients must be post-menopausal or sterilized or must not be breastfeeding, have a negative pregnancy test, have no intention to become pregnant during the study and use acceptable methods of contraception or abstain from heterosexual intercourse from Screening until 10 weeks after last dose of study drug
  • Patients must be either on riluzole for at least 30 days prior to screening or have not taken riluzole for at least 30 days prior to screening and not planning to start riluzole during the course of the study.
  • Patients on edaravone must have completed at least 2 cycles of dosing with edaravone at the time of screening or have not taken edaravone for at least 30 days prior to screening and not planning to start edaravone during the course of the study.
Exclusion Criteria
  • At the time of screening, any use of non-invasive ventilation (NIV), e.g. continuous positive airway pressure [CPAP], noninvasive bi-level positive airway pressure [NPPV] or noninvasive volume ventilation [NVV] for any portion of the day, or mechanical ventilation via tracheostomy, or on any form of oxygen supplementation
  • Neurological impairment due to a condition other than ALS
  • Presence at screening of any medically significant cardiac, pulmonary, GI, musculoskeletal, or psychiatric illness that might interfere with the patient's ability to comply with study procedures or that might confound the interpretation of clinical safety or efficacy data
  • Has taken any investigational study drug within 30 days or five half-lives of the prior agent, whichever is longer, prior to dosing
  • Known to have received CK-2127107 or tirasemtiv in any previous clinical trial
  • Has received or is considering receiving during the course of the study any form of stem cell therapy for the treatment of ALS
  • Has received or is considering receiving during the course of the study any form of gene therapy for the treatment of ALS
  • Has received or is considering obtaining during the course of the study a diaphragmatic pacing system
  • History of substance abuse within the past 2 years
  • Use of certain medications

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Reldesemtiv 450 mg twice dailyReldesemtivPatients in this arm took 3 reldesemtiv 150 mg oral tablets every 12 hours for 12 weeks.
PlaceboPlaceboPatients in this arm took 3 placebo oral tablets every 12 hours for 12 weeks.
Reldesemtiv 150 mg twice dailyReldesemtivPatients in this arm took 1 reldesemtiv 150 mg oral tablet and 2 matching placebo tablets every 12 hours for 12 weeks.
Reldesemtiv 300 mg twice dailyReldesemtivPatients in this arm took 2 reldesemtiv 150 mg oral tablets and 1 matching placebo tablet every 12 hours for 12 weeks.
Primary Outcome Measures
NameTimeMethod
Change From Baseline to Week 12 in the Percent Predicted Slow Vital Capacity (SVC)Baseline to Week 12

Slow vital capacity was measured using a spirometer (in units of liters). Following 3 to 5 breaths at rest, patients were instructed to take as deep an inspiration as possible followed by a maximum exhalation (blowing out all the air in their lungs). Values obtained were converted to percent predicted values using the Global Lung Initiative equation (ie, the test result as a percent of predicted values for patients of similar demographic and baseline characteristics \[eg, height, age, sex\]).

Secondary Outcome Measures
NameTimeMethod
Change From Baseline to Week 12 in the ALS Functional Rating Scale - Revised (ALSFRS-R) Total ScoreBaseline to Week 12

The ALSFRS-R is used to measure the progression and severity of disability in patients with ALS. The ALSFRS-R consists of 12 questions, assessing a patient's capability and independence in functional activities relevant to ALS, categorized in the following 4 domains: gross motor tasks, fine motor tasks, bulbar functions, and respiratory function. Each question is scored from 0 (indicating incapable or dependent) to 4 (normal). The total score ranges from 0 to 48. Higher scores reflect more normal function and lower scores reflect more impaired function.

Slope of Muscle Strength Mega-score From Baseline to Week 12Baseline to Week 12

A hand-held dynamometer, with a scale of 0 to 300 pounds, was used to measure muscle strength and handgrip strength (bilateral). The muscle groups tested were: elbow flexion, wrist extension, first dorsal interosseous, hip flexion, knee extension, and ankle dorsiflexion; all muscle groups were evaluated bilaterally. For each postbaseline assessment of muscle strength, the percent change from baseline was calculated for each muscle group and handgrip strength, using the following equation: (\[postbaseline value - baseline value\] / baseline value) × 100. The muscle-strength mega-score was calculated as the average of the changes (ie, percent change from baseline) observed for each of the muscle groups as well as handgrip strength. For this endpoint, negative values indicate a decline in muscle strength.

Trial Locations

Locations (63)

Washington University School of Medicine

🇺🇸

Saint Louis, Missouri, United States

Neurology Associates, P.C.

🇺🇸

Lincoln, Nebraska, United States

West Virginia University, Dept. of Neurology

🇺🇸

Morgantown, West Virginia, United States

Flinders Medical Centre

🇦🇺

Bedford Park, South Australia, Australia

Centre de recherche du Centre Hospitalier de l'Universite de Montreal

🇨🇦

Montréal, Quebec, Canada

Saskatoon City Hospital

🇨🇦

Saskatoon, Saskatchewan, Canada

CHU de Quebec-Universite Laval, Hopital de l'Enfant Jesus

🇨🇦

Quebec, Canada

Royal Brisbane and Women's Hospital

🇦🇺

Herston, Queensland, Australia

George Washington University Medical Faculty Associates

🇺🇸

Washington, District of Columbia, United States

Temple University School of Medicine

🇺🇸

Philadelphia, Pennsylvania, United States

Johns Hopkins University - Outpatient Center

🇺🇸

Baltimore, Maryland, United States

Neurological Institute, Columbia University Medical Center

🇺🇸

New York, New York, United States

SUNY Upstate Medical University

🇺🇸

Syracuse, New York, United States

Penn State Milton S. Hershey Medical Center

🇺🇸

Hershey, Pennsylvania, United States

Cedars-Sinai Medical Center

🇺🇸

Los Angeles, California, United States

Emory Clinic

🇺🇸

Atlanta, Georgia, United States

Hospital For Special Surgery

🇺🇸

New York, New York, United States

Hospital for Special Care

🇺🇸

New Britain, Connecticut, United States

Cleveland Clinic

🇺🇸

Cleveland, Ohio, United States

Saint Louis University, Department of Neurology

🇺🇸

Saint Louis, Missouri, United States

University of Massachusetts Memorial Medical Center/University of Massachusetts Medical School

🇺🇸

Worcester, Massachusetts, United States

Brain and Mind Centre, The University of Sydney

🇦🇺

Camperdown, New South Wales, Australia

University of Washington Medical Center

🇺🇸

Seattle, Washington, United States

Department of Neurology, Westmead Hospital

🇦🇺

Westmead, New South Wales, Australia

London Health Sciences Centre University Hospital

🇨🇦

London, Ontario, Canada

Montreal Neurological Institute and Hospital

🇨🇦

Montreal, Quebec, Canada

Beaumont Hospital

🇮🇪

Dublin, Ireland

IU Health Neuroscience Center of Excellence

🇺🇸

Indianapolis, Indiana, United States

The Perron Institute for Neurological and Translation Science

🇦🇺

Nedlands, Western Australia, Australia

University of Florida

🇺🇸

Gainesville, Florida, United States

Forbes Norris MDA/ALS Research Center

🇺🇸

San Francisco, California, United States

University of Colorado Hospital Anschutz Outpatient Pavilion

🇺🇸

Aurora, Colorado, United States

Mayo Clinic

🇺🇸

Rochester, Minnesota, United States

Carol & Frank Morsani Center for Advanced Healthcare - University of South Florida

🇺🇸

Tampa, Florida, United States

Henry Ford Health System

🇺🇸

Detroit, Michigan, United States

Oregon Health & Science University

🇺🇸

Portland, Oregon, United States

Vanderbilt University Medical Center - Clinical Research Center

🇺🇸

Nashville, Tennessee, United States

UTHSCSA Medical Arts and Research Center

🇺🇸

San Antonio, Texas, United States

Froedtert Memorial Lutheran Hospital

🇺🇸

Milwaukee, Wisconsin, United States

Duke Neurological Disorders Clinic

🇺🇸

Durham, North Carolina, United States

Houston Methodist Hospital

🇺🇸

Houston, Texas, United States

University of Vermont Medical Center

🇺🇸

Burlington, Vermont, United States

St. Joseph's Hospital and Medical Center - Barrow Neurological Clinics

🇺🇸

Phoenix, Arizona, United States

Stanford Hospital and Clinics

🇺🇸

Stanford, California, United States

University of California Irvine

🇺🇸

Orange, California, United States

Texas Neurology

🇺🇸

Dallas, Texas, United States

Duchossois Center for Advanced Medicine

🇺🇸

Chicago, Illinois, United States

University of Iowa Hospitals and Clinics

🇺🇸

Iowa City, Iowa, United States

Hennepin County Medical Center

🇺🇸

Minneapolis, Minnesota, United States

The Ohio State University Wexner Medical Center

🇺🇸

Columbus, Ohio, United States

Providence Brain and Spine Institute ALS Center

🇺🇸

Portland, Oregon, United States

Neurosciences Institute, Neurology - Charlotte

🇺🇸

Charlotte, North Carolina, United States

University of Virginia Health System

🇺🇸

Charlottesville, Virginia, United States

University of Calgary, Heritage Medical Research Center

🇨🇦

Calgary, Alberta, Canada

McMaster University Medical Centre

🇨🇦

Hamilton, Ontario, Canada

Sunnybrook Health Science Centre

🇨🇦

Toronto, Ontario, Canada

Hospital San Rafael Servicio de Neurologia

🇪🇸

Madrid, Spain

Michigan Medicine

🇺🇸

Ann Arbor, Michigan, United States

Wake Forest School of Medicine

🇺🇸

Winston-Salem, North Carolina, United States

VCU Health - Ambulatory Care Center (ACC)

🇺🇸

Richmond, Virginia, United States

Edmonton Kaye Clinic

🇨🇦

Edmonton, Alberta, Canada

University of Kansas Medical Center

🇺🇸

Kansas City, Kansas, United States

University Medical Center Utrecht

🇳🇱

Utrecht, Netherlands

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