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Dose Titration Study to Test Safety and Effects of CK-2017357 in Patients With Amyotrophic Lateral Sclerosis (ALS)

Phase 2
Completed
Conditions
Amyotrophic Lateral Sclerosis
Interventions
Registration Number
NCT01486849
Lead Sponsor
Cytokinetics
Brief Summary

A Phase II, double-blind, randomized, placebo-controlled ascending dose titration study to evaluate the safety, tolerability, pharmacokinetics and pharmacodynamic effects of multiple ascending doses of CK-2017357 to an individual patient maximum tolerated dose (MTD), using a within-patient twice daily (BID) dose-titration regimen in ALS patients on 50 mg riluzole once daily (QD).

Detailed Description

Patients will be randomized to one of two dosing groups, active CK-2017357 or placebo, in a 3:1 ratio. Prior to study drug dosing, patients will be required to decrease their riluzole dose to 50 mg QD for 7 days; after this 7 day period patients will either receive placebo or start the titration on active CK-2017357 while continuing to take riluzole at 50 mg QD.

Potential patients will be screened to assess their eligibility to enter the study within 21 days prior to Day -7, when they will begin taking riluzole at the decreased dose of 50 mg QD. Patients will be randomized in a 3:1 ratio to CK-2017357 (Group 1) or placebo (Group 2). On Day 1, patients will begin taking a total daily dose of 250 mg (125 mg BID) of CK-2017357 or matching placebo tablets BID for 7 days. Then they will take a total daily dose of 375 mg (125 mg morning \[AM\] and 250 mg evening \[PM\]) of CK-2017357 or matching placebo tablets BID for 7 days, and finally, they will take a total daily dose of 500 mg (250 mg BID) of CK-2017357 or matching placebo tablets BID for 7 days. A final dose of 250 mg of CK-2017357 or placebo will be taken in the morning on Day 22 at the study site.

Dose-escalation of CK-2017357 or placebo may be stopped, or the dose reduced to a lower level, based on tolerability. All patients who return to a lower dose will stay on that dose for the remainder of the study.

Patients will remain on the decreased dose of riluzole until the follow-up visit approximately 7 days after Day 22.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
27
Inclusion Criteria
  1. Able to comprehend and willing to sign an Informed Consent Form (ICF)
  2. Males or females 18 years of age or older
  3. A diagnosis of familial or sporadic ALS (defined as meeting the possible, laboratory-supported probable, probable, or definite criteria for a diagnosis of ALS according to the World Federation of Neurology El Escorial criteria)
  4. Maximum voluntary grip strength in at least one hand between 10 & 40 pounds (females) and 10 & 60 pounds (males)
  5. Able to swallow tablets with water
  6. Currently taking and tolerating a stable dose of 50 mg BID riluzole
  7. Willing and able to reduce daily dose of riluzole to 50mg QD for 5 weeks
  8. Not currently taking or willing and able to remain off theophylline-containing medications during study participation
  9. Patient has a caregiver who is capable of observing and reporting patient status
  10. Upright Slow Vital Capacity (SVC) >50% of predicted for age, height, and sex
  11. Able to perform pulmonary function tests
Exclusion Criteria
  1. Life expectancy <3 months
  2. Receipt of investigational study drug within 30 days or 5 half-lives of the prior agent, whichever is greater, prior to dosing
  3. Any prior treatment with CK-2017357
  4. Any use of non-invasive positive pressure ventilation (NIPPV), such as Continuous Positive Airway Pressure (CPAP) or Bilevel Positive Airway Pressure (BiPAP)

Other protocol-defined inclusion/exclusion criteria may apply.

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
Matching Placebo (Group 2)Riluzole 50 MGPlacebo as add-on therapy to riluzole
Dose Titration of CK-2017357 (Group 1)Riluzole 50 MGDose titration of active drug as add-on therapy to riluzole
Matching Placebo (Group 2)PlaceboPlacebo as add-on therapy to riluzole
Dose Titration of CK-2017357 (Group 1)CK-2017357Dose titration of active drug as add-on therapy to riluzole
Primary Outcome Measures
NameTimeMethod
Number of participants with adverse eventsapproximately 29 days
Secondary Outcome Measures
NameTimeMethod
Change from baseline in scores on tests of maximum handgrip strength and handgrip fatigue22 days

Measured using the DynEx Electronic Hand Dynamometer. Patients asked to squeeze the device with the maximum possible force to establish the maximum voluntary contraction. Handgrip fatigue is then measured. Patient is asked to squeeze the device until they can no longer stay above 60% of target or 120 seconds. This will be measured at Screening, Day -7, Day 1, Day 15 and Day 22.

Change from baseline in scores on tests of Sniff Nasal Inspiratory Pressure (SNIP)22 days

SNIP will be measured using the Micro Medical Respiratory Pressure Meter (MicroRPM) at Screening, Day -7, Day 1, Day 15 and Day 22.

Change from baseline in scores on tests of Maximum Voluntary Ventilation (MVV)21 days

MVV will be measured using the EasyOne Spirometer System at Screening, Day -7, Day 1, Day 15 and Day 22.

Change from baseline in Patient Global Assessment22 days

Patients will be asked to assess whether they feel the same, better or worse as compared to how they felt at pre-dose on Day 1

Change from baseline in Investigator Global Assessment22 days

Investigator will assess whether the patient appears the same, better or worse as compared to the patient's status at pre-dose on Day 1.

Change from baseline in score on the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R)22 days

An instrument for evaluating the functional status of patients with ALS. Minimum score is 0 and maximum score is 40. The higher the score the more function is retained. This will be administered at Screening, Day -7, Day 1, Day 15 and Day 22.

Change from baseline in scores on tests of Slow Vital Capacity (SVC)22 days

SVC will be measured using the ndd EasyOne Spirometer System at Screening, Day -7, Day 1, Day 15 and Day 22.

Evaluate the pharmacokinetics of CK-2017357Day 1, Day 15, and Day 22

Plasma levels of CK-2017357 will be measured at pre-dose, and at 2 and 4 hours post AM dose

Evaluate the pharmacokinetics of riluzole in patients receiving CK-2017357Day 1, Day 15, and Day 22

Plasma levels of riluzole will be measured at pre-dose and at 2 and 4 hours post AM dose

Change from baseline in scores on tests of muscle strength22 days

Muscle strength is measured using Hand Held Dynamometry. A series of assessments are done on different muscle groups. This will be measured at Day -7, Day 1, and Day 22.

Change from baseline in scores on tests of Timed Up and Go22 days

TUG is measured by timing how long it takes for a subject to stand up from a chair, walk 10 feet, turn around, walk back to the chair and sit down. This will be measured at Day -7, Day 1, and Day 22.

Trial Locations

Locations (11)

University of California at Irvine, ALS and Neuromuscular Center

🇺🇸

Orange, California, United States

Coordinated Clinical Research

🇺🇸

La Jolla, California, United States

Massachusetts General Hospital, Neurology Clinical Trials Unit

🇺🇸

Charlestown, Massachusetts, United States

Washington University

🇺🇸

Saint Louis, Missouri, United States

Cornell Faculty, Hospital for Special Surgery

🇺🇸

New York, New York, United States

Providence ALS Center

🇺🇸

Portland, Oregon, United States

University of California at San Francisco, Fresno Campus, Central California Neurological Institute

🇺🇸

Fresno, California, United States

Duke University School of Medicine, Division of Neurology

🇺🇸

Durham, North Carolina, United States

Ohio State University, Department of Neurology

🇺🇸

Columbus, Ohio, United States

University of Texas Health Science Center, Department of Neurology

🇺🇸

San Antonio, Texas, United States

Hospital for Special Care

🇺🇸

New Britain, Connecticut, United States

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