Actively Recruiting

Age: 2Years +
All Genders
ID06078553

Multicenter, Multinational, Natural History Study in Participants With Congenital Myasthenic Syndromes Due to Mutations in DOK7, MUSK, AGRN, or LRP4

Led by argenx · Updated on 2026-06-03

100

Participants Needed

30

Research Sites

N/A

Total Duration

On this page

AI-Summary

What this Trial Is About

This research aims to understand disease activity in patients with Congenital Myasthenic Syndromes (CMS) caused by mutations in the genes DOK7, MUSK, AGRN, or LRP4. It will evaluate symptoms and quality of life in these patients to gather important information about their condition. The study is observational and collects both retrospective and prospective data to better comprehend CMS. Participants will attend up to four study visits where clinical assessments will be performed. These assessments include evaluating changes in health status related to CMS and collecting data on diagnosis, healthcare use, and medications over a period of up to 12 months. Researchers will also monitor various scores related to muscle strength, daily living activities, fatigue, and quality of life. During the study, participants will undergo evaluations such as the Quantitative Myasthenia Gravis score, MG-ADL, PROMIS-GHS, PROMIS-DFL, EQ-5D-5L, 3TUG, and Neuro-QoL Fatigue score to measure their symptoms and well-being. The study aims to summarize these outcomes and track changes over a year. Participation involves attending scheduled visits for assessments and providing clinical information to help researchers understand CMS progression better.

CONDITIONS

Brief Title

A Natural History Study in Participants With Congenital Myasthenic Syndromes (CMS) Due to Mutations in DOK7, MUSK, AGRN, or LRP4

Who Can Participate

Age: 2Years +
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Can understand the study and provide written informed consent or assent
  • Male or female aged 2 years or older at consent
  • Diagnosis of CMS due to biallelic pathogenic mutations in DOK7 or any pathogenic mutations in MUSK, AGRN, or LRP4
  • Total Quantitative Myasthenia Gravis (QMG) score of 3 or higher (for participants aged 6 years or older)
  • For those taking oral beta agonists (e.g., albuterol, salbutamol, ephedrine), must have been on medication for at least 3 months before screening
Not Eligible

You will not qualify if you...

  • Any medical condition that might interfere with accurate CMS assessment, as judged by the investigator
  • Currently participating in any interventional clinical study with a study drug
  • Diagnosis of CMS due to mutations in genes other than DOK7, MUSK, AGRN, or LRP4

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)

Monitoring

Duration - Up to 12 months

Participants are observed to collect data on diagnosis, health care utilization, medications, and changes in health status related to Congenital Myasthenic Syndromes (CMS).

Periodic visits for data collection over 12 months

Trial Site Locations

Total: 30 locations

1

UC Davis Health - UC Davis Health Midtown Ambulatory Care Center

Sacramento, California, United States, 95816

Actively Recruiting

2

Ann and Robert H Lurie Children's Hospital of Chicago - Main Hospital

Chicago, Illinois, United States, 60611

Actively Recruiting

3

Hospital Sisters Health System (HSHS) - St Elizabeth's Hospital

O'Fallon, Illinois, United States, 62269

Actively Recruiting

4

Harvard Medical School - Boston Children's Hospital

Boston, Massachusetts, United States, 02115

Actively Recruiting

5

Ohio State Martha Moorehouse

Columbus, Ohio, United States, 43221

Actively Recruiting

6

The Children's Hospital of Philadelphia (CHOP)

Philadelphia, Pennsylvania, United States, 19104

Actively Recruiting

7

Medical University of Vienna

Vienna, Austria, 1090

Actively Recruiting

8

Hopital Universitaire de Bruxelles/ Academisch Ziekenhuis Brussel

Brussels, Anderlecht, Belgium, 1070

Actively Recruiting

9

Pseg Centro De Pesquisa ClinicaS.A

São Paulo, Brazil, 04038-002

Actively Recruiting

10

The Children's Hospital of Eastern (CHEO), Ottawa

Ottawa, Ontario, Canada, K1H8L1

Actively Recruiting

11

The Ottawa Hospital - Civic Campus

Ottawa, Canada, K1Y 4E9

Actively Recruiting

12

CHU Bordeaux - Groupe Hospitalier Pellegrin - Neurology

Bordeaux, France, 33076

Actively Recruiting

13

Hospices Civils de Lyon (HCL) - Hopital Pierre Wertheimer - Neurology

Bron, France, 69500

Actively Recruiting

14

CHU Lille Hopital Salengro - Neurology

Lille, France, 59037

Actively Recruiting

15

CHU Timone

Marseille, France, 13385

Actively Recruiting

16

Assistance Publique Hopitaux de Paris - Hopital Pitie-Salpetriere

Paris, France, 75013

Actively Recruiting

17

Centre Hospitalier Regional Universitaire de Strasbourg - Hopital de Hautepierre

Strasbourg, France, 67000

Actively Recruiting

18

Charite - Universitaetsmedizin Berlin

Berlin, Germany, 10117

Actively Recruiting

19

UKRUB - Berufsgenossenschaftliches Universitatsklinikum Bergmannsheil GmbH

Bochum, Germany, 44789

Actively Recruiting

20

Universitaetsklinikum Essen (AoR)

Essen, Germany, 45147

Actively Recruiting

21

Fondazione IRCCS Istituto Neurologico Carlo Besta

Milan, Italy, 20133

Actively Recruiting

22

National Center Hospital, National Center of Neurology and Psychiatry

Kodaira, Japan, 187-8551

Actively Recruiting

23

National Hospital Organization Akita Hospital - Neurology

Yurihonjō, Japan, 018-1393

Actively Recruiting

24

Medicover Integrated Clinical Services (MICS) Centrum Medyczne Bydgoszcz

Bydgoszcz, Poland, 85-065

Actively Recruiting

25

Centrum Medyczne Neurologia Slaska - Neurology

Katowice, Poland, 40-689

Actively Recruiting

26

Hospital Sant Joan de Deu - Pediatric Neurology

Esplugues de Llobregat, Spain, 08950

Actively Recruiting

27

Hospital Universitario 12 de Octubre

Madrid, Spain, 28041

Actively Recruiting

28

Hospital Universitari i Politecnic La Fe

Valencia, Spain, 46026

Actively Recruiting

29

Ulster Hospital

Dundonald, United Kingdom, BT16 1RH

Actively Recruiting

30

Oxford University Hospitals NHS Foundation Trust - John Radcliffe Hospital

Oxford, United Kingdom, OX3 9DU

Actively Recruiting

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Research Team

S

Sabine Coppieters, MD

How is the study designed?

Study Type

OBSERVATIONAL

Masking

N/A

Allocation

N/A

Model

N/A

Primary Purpose

N/A

Number of Arms

0

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