Recruiting

Observational Study

Sponsor:

NIH

Code:

NCT06287762

Conditions

Ryanodine Receptor 1-Related Myopathy

Ryanodine Receptor 1 Related Disorders

Eligibility Criteria

Sex: All

Age: 7 - 70+

Healthy Volunteers: Not accepted

Study Details

Brief summary:

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

Ryanodine Receptor 1-Related Myopathy

Ryanodine Receptor 1 Related Disorders

Study ID

NCT06287762

Start date

Mar 11, 2025

Status verified date

Jan 21, 2026

Completion date

Dec 30, 2031

Anticipated

Primary completion date

Apr 30, 2031

Anticipated

Eligibility Criteria

Eligibility Criteria

Sex: All

Age: 7 - 70+

Healthy Volunteers: Not accepted

  • INCLUSION CRITERIA (CENTRALIZED ARM)

1. Stated willingness to comply with all study procedures, availability for the duration of the study, and submission of medical records to research team prior to screening.
2. Male or female, aged >=7 years of age.
3. Genetically confirmed RYR1-related disorder, evidenced by pathogenic or likely pathogenic variants identified by CLIA testing (whole genome, exome, targeted, partial or full RYR1 sequencing) OR variant of uncertain significance with supporting clinical phenotype.
4. Agreement to adhere to Lifestyle Considerations throughout study duration.
5. Ability of subject to communicate their understanding of the purpose of the study, and willingness to provide assent and/or to sign a written informed consent document.
6. Resides in the United States.

EXCLUSION CRITERIA (CENTRALIZED ARM)

1. Participation in an IND, IDE, or equivalent clinical study in the past six months
2. Severe disability or mobility issues (inability to walk 10 meters with or without assistance)
3. Requires mechanical ventilation or tracheotomy
4. Other neuromuscular diseases resulting in muscle weakness
5. Ongoing medical condition that is deemed by the Principal Investigator to interfere with the conduct or assessments of the study (e.g. active infection) or safety of the subject.

INCLUSION CRITERIA (DE-CENTRALIZED ARM)

1. Stated willingness to comply with all study procedures, availability for the duration of the study, and submission of medical records to research team prior to screening.
2. Male or female, aged > 7 years of age.
3. Genetically confirmed RYR1-related disorder, evidenced by pathogenic or likely pathogenic variants identified by CLIA testing (whole genome, exome, targeted, partial or full RYR1 sequencing) OR variant of uncertain significance with supporting clinical phenotype.
4. Ability of subject to communicate their understanding of the purpose of the study, and willingness to provide assent and/or sign a written informed consent document.
5. Resides in the United States

EXCLUSION CRITERIA (DE-CENTRALIZED ARM)

1. Participation in an IND, IDE, or equivalent clinical study in the past six months
2. Other neuromuscular diseases resulting in muscle weakness
3. Ongoing medical condition that is deemed by the Principal Investigator to interfere with the conduct or assessments of the study (e.g. active infection) or safety of the subject

Study Design

Enrollment

150 participants

Anticipated

Interventions and Outcome Measures

Arms

Centralized

Visits are conducted at the NIH clinical center. All participants are ambulatory.

Decentralized

Visits are conducted via telehealth.

Primary outcome measure

  • Adverse and disease-related events [ Time Frame: 3 years ]
  • Medical data review [ Time Frame: 3 years ]
  • Ophthalmology [ Time Frame: 3 years ]
  • Patient-reported outcomes [ Time Frame: 3 years ]
  • Pulmonary function [ Time Frame: 3 years ]
  • Motor function and performance [ Time Frame: 3 years ]

Central Contacts and Locations

Central contacts

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

(301) 451-5951lawalt@mail.nih.gov

Locations

National Institutes of Health Clinical Center

Recruiting

Bethesda, Maryland, United States, 20892

Contacts

NIH Clinical Center Office of Patient Recruitment (OPR)

(800) 411-1222ccopr@nih.gov

More Information

Sponsor

National Institutes of Health Clinical Center (CC)

Last update posted

Jan 23, 2026

Last verified

Jan 21, 2026

Keywords

  • RYR1
  • Muscle Disease
  • Natural History
  • Congenital Myopathy

Trial information was received from ClinicalTrials.gov and was last updated on 2026-09-10. This information was provided to ClinicalTrials.gov by National Institutes of Health Clinical Center (CC) on 2026-01-23.