A Study of the Natural History of Participants With LGMD2E/R4, LGMD2D/R3, LGMD2C/R5, and LGMD2A/R1 ≥ 4 Years of Age, Who Are Managed in Routine Clinical Practice

NCT ID: NCT04475926

Last Updated: 2025-11-06

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

ACTIVE_NOT_RECRUITING

Total Enrollment

205 participants

Study Classification

OBSERVATIONAL

Study Start Date

2021-04-22

Study Completion Date

2030-09-05

Brief Summary

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This study will follow participants who are screened and confirmed with a genetic diagnosis of Limb-girdle muscular dystrophy type 2E (LGMD2E/R4), Limb-girdle muscular dystrophy type 2D (LGMD2D/R3), Limb-girdle muscular dystrophy type 2C (LGMD2C/R5), or Limb-girdle muscular dystrophy type 2A (LGMD2A/R1).

These enrolled participants will be followed to evaluate mobility and pulmonary function for up to 5 years after enrollment for participants with LGMD2C/R5, LGMD2D/R3, and LGMD2E/R4 with a North Star Assessment for Dysferlinopathy (NSAD) ≥ 25 at Baseline, up to 3 years for participants with LGMD2C/R5, LGMD2D/R3, and LGMD2E/R4 with a NSAD \< 25 at Baseline, and up to 3 years for participants with LGMD2A/R1. Additional participant data will be collected from the time the individual began experiencing LGMD symptoms to the present.

Detailed Description

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LGMD Type 2A, 2C, and 2D cohorts are now closed. Enrollment in the LGMD Type 2E cohort is closed and participants will be followed for up to five years.

Conditions

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Limb-girdle Muscular Dystrophy

Keywords

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North Star Assessment for Dysferlinopathy (NSAD) Performance of Upper Limb (PUL) Pulmonary function tests (PFTs) Ambulatory Non-Ambulatory Limb-girdle LGMD sarcoglycanopathy β -sarcoglycan Muscular Dystrophy α -sarcoglycan γ -sarcoglycan LGMD-2D/R3 LGMD-2E/R4 LGMD-2C/R5 LGMD2A/R1 Clinical Outcomes Assessment

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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LGMD2E/R4 Cohort

Participants with LGMD2E/R4 will be enrolled in this cohort. Enrollment will be capped according to age as follows: 4 to 7 years age range, 8 to 16 years age range, and ≥17 years age range through the course of the study.

No interventions assigned to this group

LGMD2D/R3 Cohort

Participants with LGMD2D/R3 will be enrolled in this cohort. Enrollment will be capped according to age as follows: 4 to 7 years age range, 8 to 16 years age range, and ≥17 years age range through the course of the study.

No interventions assigned to this group

LGMD2C/R5 Cohort

Participants with LGMD2C/R5 will be enrolled in this cohort. Enrollment will be capped according to age as follows: 4 to 7 years age range, 8 to 16 years age range, and ≥17 years age range through the course of the study.

No interventions assigned to this group

LGMD2A/R1 Cohort

Participants with LGMD2A/R1 will be enrolled in this cohort. Enrollment will be capped according to age as follows: 4 to 7 years age range, 8 to 16 years age range, and ≥17 years age range through the course of the study.

No interventions assigned to this group

Eligibility Criteria

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Inclusion Criteria

* Male or female participant ≥ 4 years of age who demonstrate symptoms of LGMD2E/R4, LGMD2D/R3, LGMD2C/R5, or LGMD2A/R1 in the opinion of the investigator (eg, muscle weakness, loss of function, delayed milestones).
* Confirmed clinical and genetic diagnosis of LGMD2E/R4, LGMD2D/R3, LGMD2C/R5, or LGMD2A/R1.

Exclusion Criteria

* Demonstrates cognitive delay or impairment that could confound motor development, in the opinion of the Investigator.
* Has a medical condition, in the opinion of the Investigator, that might compromise participants ability to comply with study requirements.
* Is participating in other interventional study(ies) at the time of enrollment in this study.
Minimum Eligible Age

4 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

No

Sponsors

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Sarepta Therapeutics, Inc.

INDUSTRY

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Medical Director

Role: STUDY_DIRECTOR

Sarepta Therapeutics, Inc.

Locations

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Barrow Neurological Institute

Phoenix, Arizona, United States

Site Status

Arkansas Children's

Little Rock, Arkansas, United States

Site Status

University of California San Diego

La Jolla, California, United States

Site Status

University of California, Davis Health Dept of PM&R

Sacramento, California, United States

Site Status

Anne & Robert H. Lurie Children's Hospital of Chicago

Chicago, Illinois, United States

Site Status

Nationwide Children's Hospital

Columbus, Ohio, United States

Site Status

Oregon Health and Science University

Portland, Oregon, United States

Site Status

The Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status

University of Texas Southwestern Medical Center

Dallas, Texas, United States

Site Status

University of Utah Hospital

Salt Lake City, Utah, United States

Site Status

Children's Hospital of the King's Daughters

Norfolk, Virginia, United States

Site Status

University Hospital Gent

Ghent, Oost-Vlaanderen, Belgium

Site Status

UZ Leuven

Leuven, Vlaams Brabant, Belgium

Site Status

Hôpital Universitaire des Enfants Reine Fabiola

Brussels, , Belgium

Site Status

Hospital de Clinicas de Porto Alegre (HCPA) - PPDS

Porto Alegre, Rio Grande do Sul, Brazil

Site Status

Children's Hospital - London Health Science Centre

London, Ontario, Canada

Site Status

Zentrum für Kinderheilkunde und Jugendmedizin Uniklinikum Giessen Marburg (UKGM), Standort Giessen

Giessen, Hesse, Germany

Site Status

Universitätsklinikum Essen

Essen, North Rhine-Westphalia, Germany

Site Status

Istituto Giannina Gaslini

Genoa, Liguria, Italy

Site Status

Azienda Ospedale Università Padova

Padua, Veneto, Italy

Site Status

Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico

Milan, , Italy

Site Status

Hospital Sant Joan de Déu Universidad de Barcelona

Barcelona, , Spain

Site Status

Karolinska Universitetssjukhuset Solna

Stockholm, Stockholm County, Sweden

Site Status

Lokman Hekim Etlik Hastanesi

Ankara, , Turkey (Türkiye)

Site Status

UCL Institute of Child Health & Great Ormond Street Hospital for Children

London, , United Kingdom

Site Status

Institute of Genetic Medicine, International Centre for Life

Newcastle upon Tyne, , United Kingdom

Site Status

Countries

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United States Belgium Brazil Canada Germany Italy Spain Sweden Turkey (Türkiye) United Kingdom

Other Identifiers

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SRP-LGMD-501-NHS

Identifier Type: -

Identifier Source: org_study_id