Actively Recruiting

Age: 0 - 59Months
All Genders
ID05224778

Assessing Pediatric Endpoints in DM1 (ASPIRE-DM1)

Led by Virginia Commonwealth University · Updated on 2025-06-11

50

Participants Needed

5

Research Sites

8 weeks

Total Duration

On this page

AI-Summary

What this Trial Is About

This research aims to establish reliable clinical measures and biomarkers for children with congenital myotonic dystrophy type 1 (CDM), a genetic muscle disorder that begins at birth. CDM affects infants with symptoms like low muscle tone, feeding problems, or breathing difficulties requiring hospital care. The study focuses on understanding developmental milestones, physical and cognitive abilities, and quality of life in a larger group of affected children than previous studies. Participants are children from newborn to 3 years and 11 months old diagnosed with CDM, confirmed by genetic testing showing a specific gene repeat expansion. The study observes these children without providing experimental treatments. Researchers track their motor milestones and other functions over time using assessments like the Vineland, dysarthria evaluation, and gross motor function measure, comparing results with typically developing children. During the study, children will undergo various developmental and functional tests through 18 months to evaluate motor skills and other abilities. Parents or guardians will provide consent and help with study procedures. The researchers will collect information on motor milestone attainment and other developmental measures, aiming to correlate these with potential biomarkers. The study will continue until the primary outcomes are assessed at 18 months.

CONDITIONS

Brief Title

DMCRN-02-001: Assessing Pediatric Endpoints in DM1

Who Can Participate

Age: 0 - 59Months
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Age from newborn to 3 years 11 months at enrollment
  • Diagnosis of congenital myotonic dystrophy with symptoms starting within 30 days of birth
  • Symptoms include low muscle tone, feeding or breathing difficulties requiring hospitalization for more than 72 hours
  • Genetic test confirming expanded CTG repeat in DMPK gene in child or mother (greater than 200 repeats or E1-E4 classification)
  • Guardian willing and able to sign consent and follow study procedures
Not Eligible

You will not qualify if you...

  • Any other illness that could interfere with study participation or results
  • Significant trauma within the past month
  • Presence of internal metal or devices (for DEXA component exclusion)
  • History of bleeding disorder or platelet count below 50,000
  • History of allergic reaction to local anesthetic

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Your Study Journey

Screening

Duration - 2 to 4 weeks

Participants are screened for eligibility to participate in the trial.

1 visit (in-person)

Long-term Monitoring

Duration - Up to 18 months

Participants are observed over time to evaluate motor milestone attainment, motor functions, cognitive function, and quality of life measures up to 18 months.

Regular visits over the 18-month period

Trial Site Locations

Total: 5 locations

1

University of California, Los Angeles

Los Angeles, California, United States, 90095

Actively Recruiting

2

University of Kansas Medical Center

Fairway, Kansas, United States, 66205

Actively Recruiting

3

University of Rochester Medical Center

Rochester, New York, United States, 14642

Actively Recruiting

4

Virginia Commonwealth University

Richmond, Virginia, United States, 23298

Actively Recruiting

5

Centro Clinico NeMO

Milan, Italy, 20162

Actively Recruiting

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Research Team

R

Ruby Langeslay

J

Jennifer Raymond

How is the study designed?

Study Type

OBSERVATIONAL

Masking

N/A

Allocation

N/A

Model

N/A

Primary Purpose

N/A

Number of Arms

1

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