Actively Recruiting

Age: 7Years - 100Years
All Genders
NCT06287762

A Natural History Study of RYR1-Related Disorders

Led by National Institutes of Health Clinical Center (CC) · Updated on 2026-01-23

150

Participants Needed

1

Research Sites

355 weeks

Total Duration

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AI-Summary

What this Trial Is About

Background: Congenital myopathies (CM) are genetic disorders that can cause decreased muscle tone and muscle weakness. Most CMs in the United States are related to the ryanodine receptor 1 (RYR1) gene. Researchers need more natural history data to learn about these CMs in children and adults. Objective: To learn more about the signs, symptoms, and course of RYR1-related disorders. Eligibility: People aged 7 years and older with an RYR1-related disorder. Design: Ambulatory participants will come to the Clinical Center and non-ambulatory participants will visit via telehealth. Visits will be once a year for 3 or 5 years. Clinical Center visits will take 2 to 3 days. All participants will undergo tests including: Photos and videos. These will be taken to document the participant s condition. Blood and urine tests. Activity Tracker. Participants will wear a device to record their activity. Questionnaires. Participants will answer questions about their health, pain, fatigue, stress, quality of life, and other topics. Participants who visit the Clinical Center will also undergo: Tests of heart and lung function. Motor skills and strength tests. Participants will walk, climb stairs, kneel, crawl, stand up, and perform other movements to test their strength and abilities. They will squeeze and pinch a handheld device to test their grip. Imaging scans. Skin biopsy. Adult participants may opt to have a sample of skin taken (one time only). Eye exam

CONDITIONS

Official Title

A Natural History Study of RYR1-Related Disorders

Who Can Participate

Age: 7Years - 100Years
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Willingness to comply with all study procedures and provide medical records before screening
  • Male or female aged 7 years or older
  • Genetically confirmed RYR1-related disorder shown by specific genetic testing or clinical phenotype with uncertain variant
  • Agreement to follow lifestyle considerations during the study
  • Ability to understand the study purpose and provide informed consent or assent
  • Residence in the United States
Not Eligible

You will not qualify if you...

  • Participation in a clinical study with investigational drug or device in the past six months
  • Severe disability or inability to walk 10 meters with or without assistance (centralized arm only)
  • Need for mechanical ventilation or tracheotomy (centralized arm only)
  • Other neuromuscular diseases causing muscle weakness
  • Ongoing medical condition that may interfere with the study or safety, such as an active infection

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Trial Site Locations

Total: 1 location

1

National Institutes of Health Clinical Center

Bethesda, Maryland, United States, 20892

Actively Recruiting

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Research Team

I

Irene C Chrismer, R.N.

CONTACT

T

Tokunbor A Lawal, C.R.N.P.

CONTACT

How is the study designed?

Study Type

OBSERVATIONAL

Masking

N/A

Allocation

N/A

Model

N/A

Primary Purpose

N/A

Number of Arms

2

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A Natural History Study of RYR1-Related Disorders | DecenTrialz