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A Multicenter, Double-Blind Study to Investigate the Safety and Efficacy of Arimoclomol in Volunteers With ALS

Phase 2
Withdrawn
Conditions
Amyotrophic Lateral Sclerosis
Interventions
Drug: Placebo
Registration Number
NCT00561366
Lead Sponsor
CytRx
Brief Summary

Arimoclomol is a small molecule that upregulates "molecular chaperones" in cells under stress. Arimoclomol extends survival by five weeks when given both pre-symptomatically and at disease onset in a mutant superoxide dismutase (SOD1) transgenic mouse model of ALS. Furthermore, it has been demonstrated to have neuroprotective and neuroregenerative effects in other rat models of nerve damage. Molecular chaperone proteins are critical in the cellular response to stress and protein misfolding. Recent data suggest that the SOD1 mutation responsible for ALS in some patients with familial disease reduces the availability of a variety of molecular chaperones, and thus weakens their ability to respond to cellular stress. Protein misfolding and consequent aggregation may play a role in the pathogenesis of both the familial and sporadic forms of ALS. Therapeutic agents such as arimoclomol that improve cellular chaperone response to protein misfolding may be helpful in ALS.

Detailed Description

This is a Phase 2b double-blind, randomized, placebo-controlled parallel-group study evaluating the safety and efficacy of arimoclomol (400 mg t.i.d.) compared to placebo. A safety lead-in phase will be employed to ensure the safety of all study volunteers.

Tier I (Safety Lead-in): During the enrollment period for the safety lead-in phase, 24 volunteers meeting inclusion/exclusion criteria will be randomized at 4 investigative sites. These volunteers will have weekly visits during the first 4 weeks after starting treatment. Pharmacokinetics (PK) will be performed at various timepoints throughout these 4 weeks. After the initial 4 weeks of treatment, visits will continue at 4-week intervals up to Week 36, subsequently visits will occur every 8 weeks up to Week 68. A final visit will occur at Week 72. There will be a 28-day post study medication Follow-Up Telephone Call to assess medical status and adverse events.

Tier II: After the Tier I volunteers finish 4 weeks of treatment, their data will be reviewed by the IDMC and, if no serious safety issues are identified, the recommendation will be made to start the second enrollment period (Tier II). During Tier II enrollment, volunteers recruited from approximately 30 to 40 centers in the US and Canada will be randomized. After screening and randomization, volunteers will be followed every 4 weeks for 9 months. Subsequently visits will occur every 8 weeks up to Week 68, with interim Follow-Up Telephone Calls at Weeks 16, 24, and 32 and a final visit at Week 72. A Week 76 Follow-Up Telephone Call to assess medical status and adverse events will occur at 28 days post last dose of study medication.

Recruitment & Eligibility

Status
WITHDRAWN
Sex
All
Target Recruitment
Not specified
Inclusion Criteria
  • Familial or sporadic ALS.
  • Diagnosed with laboratory-supported probable, probable or definite ALS according to the World Federation of Neurology El Escorial criteria for less than or equal to 36 months' duration prior to the Screening Visit.
  • Vital capacity (VC) equal to or greater than 70% predicted value for gender, height and age at the Screening Visit.
  • Geographic accessibility to the study site.
  • Ability to take oral medication at the Screening Visit, based on verbal report.
  • Fluency in English, Spanish or Canadian French.
Exclusion Criteria
  • History of known sensitivity or intolerability to arimoclomol or to any other related compound.

  • Prior exposure to arimoclomol through a clinical trial or physician-sponsored IND.

  • Exposure to any investigational agent within 30 days of the Screening Visit.

  • Presence of any of the following clinical conditions:

    1. Substance abuse within the past year
    2. Unstable cardiac, pulmonary, renal, hepatic, endocrine, hematologic, or active malignancy or infectious disease
    3. AIDS or AIDS-related complex
    4. Unstable psychiatric illness defined as psychosis (hallucinations or delusions), untreated major depression within 90 days of the Screening Visit.
  • Laboratory values: Screening serum creatinine greater than or equal to 1.5 mg/dL, creatinine clearance less than 70 cc/min, alanine aminotransferase (ALT) greater than 3.0 times the upper limit of normal, total bilirubin greater than 1.5 times the upper limit of normal, white blood cell (WBC) count less than 3,500/mm3, platelet concentration of <100,000/ul, hematocrit level of less than 33 % for female or less than 35 % for male, or coagulation tests (PT, PTT) greater than or equal to 1.5 times upper limit of normal.

  • Female volunteers who are breast-feeding.

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
1Placebo-
2Arimoclomol-
Primary Outcome Measures
NameTimeMethod
ALSFRS-R9 months
Secondary Outcome Measures
NameTimeMethod
ALSFRS-R18 months
Survival18 months
Muscle strength9 and 18 months
Pulmonary function9 and 18 months
MUNE9 and 18 months
Quality of Life9 and 18 months

Trial Locations

Locations (35)

Virginia Mason Clinic - Tier 2 Site

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Seattle, Washington, United States

Massachusetts General Hospital - Tier 1 Site

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Boston, Massachusetts, United States

University of Kansas Medical Center - Tier 2 site

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Kansas City, Kansas, United States

Washington University - Tier 2 Site

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St. Louis, Missouri, United States

Saint Louis University, Neuromuscular Div. - Tier 2 Site

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Saint Louis, Missouri, United States

Upstate Clinical Research, LLC - Tier 2 Site

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Albany, New York, United States

Emory University - Tier 2 site

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Atlanta, Georgia, United States

John Hopkins University - Tier 2 Site

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Baltimore, Maryland, United States

University of Toronto, Sunnybrook Health Sciences Centre - Tier 2 Site

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Toronto, Ontario, Canada

University of Pittsburgh Medical Center - Tier 2 Site

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Pittsburgh, Pennsylvania, United States

Columbia University Medical Center - Tier 2 site

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New York, New York, United States

Pennsylvania State University School of Medicine - Tier 2 Site

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Hershey, Pennsylvania, United States

University of California Los Angeles - Tier 2 Site

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Pacific Palisades, California, United States

Northwestern University, Dept. of Neurology - Tier 2 Site

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Chicago, Illinois, United States

Baystate Medical Center - Tier 2 Site

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Springfield, Massachusetts, United States

University of Miami - Tier 2 Site

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Miami, Florida, United States

Mount Sinai School of Medicine - Tier 2 Site

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New York, New York, United States

Cleveland Clinic Foundation -Tier 2 site

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Cleveland, Ohio, United States

Wake Forest University School of Medicine -Tier 2 Site

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Winston Salem, North Carolina, United States

Medical College of Wisconsin - Tier 2 Site

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Milwaukee, Wisconsin, United States

University of California - San Francisco - Tier 2 Site

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San Francisco, California, United States

University of Colorado Health Sciences Center - Tier 2 Site

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Denver, Colorado, United States

Duke University Medical Center - Tier 1 Site

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Durham, North Carolina, United States

Providence ALS Center - Tier 2 Site

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Portland, Oregon, United States

University of Texas Health Science Center - Tier 2 Site

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San Antonio, Texas, United States

SUNY Downstate Medical Center - Tier 1 Site

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Syracuse, New York, United States

Drexel University College of Medicine - Tier 1 Site

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Philadelphia, Pennsylvania, United States

University of Virginia - Tier 2 Sites

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Charlottesville, Virginia, United States

Texas Neurology, PA - Tier 2 Site

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Dallas, Texas, United States

London Health Science Center - Tier 2 Site

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London, Ontario, Canada

BryanLGH Medical Center - Tier 2 Site

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Lincoln, Nebraska, United States

Vanderbilt University Medical Center - Tier 2

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Nashville, Tennessee, United States

University of Vermont, College of Medicine - Tier 2

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Burlington, Vermont, United States

University of British Columbia, Gordon and Leslie Diamond Health Care Centre - Tier 2 Site

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Vancouver, British Columbia, Canada

Montreal Neurological Institute - Tier 2

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Montreal, Quebec, Canada

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