RecruitingNCT05618080

LGMD R1 Natural History Study

GRASP-01-003: Trial Readiness and Endpoint Assessment in LGMD R1


Sponsor

Virginia Commonwealth University

Enrollment

100 participants

Start Date

Jan 31, 2024

Study Type

OBSERVATIONAL

Conditions

Summary

This is a 24-month, observational study of 100 participants with Limb Girdle Muscular Dystrophy type R1, also known as CAPN3.


Eligibility

Min Age: 12 YearsMax Age: 50 Years

Inclusion Criteria4

  • Age between 12-50 at enrollment
  • Clinically affected (defined as weakness on bedside evaluation in a pattern consistent with LGMDR1)
  • Genetic confirmation of LGMDR1 (presence of homozygous or compound heterozygous pathogenic mutations in CAPN3).
  • Must be able to provide written informed consent and be willing and able to comply with all study requirements. Note: Adult participants must be able to provide consent themselves. Legally authorized representatives are not permitted to consent on behalf of adult participants.

Exclusion Criteria5

  • Have contraindications to MRI or MRS (e.g., non-MR compatible implanted medical devices or severe claustrophobia)
  • Non-ambulatory as defined by those who are not able to walk 10 meters without assistive devices (ankle foot orthotics excluded)
  • Positive pregnancy test at any timepoint during the trial
  • Have dominantly inherited CAPN3 mutations (LGMDD4)
  • Any other illness that would interfere with the ability to undergo safe testing or would interfere with interpretation of the results in the opinion of the site investigator.

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Locations(12)

University of California, Irvine

Orange, California, United States

University of Colorado Anschutz Medical Campus

Aurora, Colorado, United States

University of Florida

Gainesville, Florida, United States

The Community Health Clinic, Inc.

Shipshewana, Indiana, United States

University of Iowa Hospitals and Clinics

Iowa City, Iowa, United States

University of Kansas Medical Center

Kansas City, Kansas, United States

University of Minnesota, Department of Neurology

Minneapolis, Minnesota, United States

Washington University School of Medicine

St Louis, Missouri, United States

Nationwide Children's Hospital

Columbus, Ohio, United States

Virginia Commonwealth University

Richmond, Virginia, United States

Leiden University Medical Center

Leiden, Netherlands

Newcastle University

Newcastle upon Tyne, United Kingdom

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NCT05618080


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