Congenital myasthenic syndrome

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ORPHA:590OMIM:617143G70.2
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4Active trials7Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Congenital myasthenic syndrome (CMS), sometimes called congenital myasthenia, is a group of rare inherited disorders that affect the way nerve signals are sent to muscles. In a healthy body, nerves and muscles communicate at a junction called the neuromuscular junction. In CMS, this communication is disrupted because of a genetic change, causing muscles to receive weak or unreliable signals. This leads to muscle weakness that is often present from birth or early childhood, though some forms appear later in life. The weakness in CMS can affect many parts of the body. The muscles around the eyes and eyelids are commonly involved, causing drooping eyelids (ptosis) and double vision. Muscles used for swallowing, breathing, and moving the arms and legs can also be affected. Symptoms often get worse with physical activity and improve with rest. Breathing problems can be serious, especially in babies and young children. There is no cure for CMS, but several medications can help manage symptoms by improving nerve-to-muscle communication. The right treatment depends on which gene is affected, so genetic testing is very important. With proper care, many people with CMS can lead active and fulfilling lives, though some forms are more severe than others.

Also known as:

Key symptoms:

Drooping eyelids (ptosis)Double visionMuscle weakness that gets worse with activityDifficulty swallowingWeak cry or feeding problems in newbornsBreathing difficulties, especially during illness or stressFatigue that comes on quickly during physical activityWeakness in the arms and legsPoor head control in infantsDelayed motor milestones such as sitting or walkingFacial weakness or expressionless faceSlurred or nasal-sounding speechScoliosis (curved spine) in some cases

Clinical phenotype terms (50)— hover any for plain English
Sudden episodic apneaHP:0002882Fatigable weaknessHP:0003473Frontalis muscle weaknessHP:0004661Intermittent episodes of respiratory insufficiency due to muscle weaknessHP:0004889Bulbar palsyHP:0001283Apneic episodes precipitated by illness, fatigue, stressHP:0002872
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

10 events
Oct 2026hiPSC-CMs-loaded Chitosan Patch to Treat Severe Chronic Ischemic Heart Disease

HELP Therapeutics Co., Ltd. — PHASE1, PHASE2

TrialNOT YET RECRUITING
Mar 2026A Multicenter, Randomized, Double-Blind, Placebo-Controlled, Phase Ⅱ Clinical Study to Evaluate the Efficacy and Safety of CMS-D001 in Adult Patients With Moderate to Severe Atopic Dermatitis

Dermavon Holdings Limited — PHASE2

TrialNOT YET RECRUITING
Mar 2026A Clinical Study Evaluating the Efficacy and Safety of CMS-D001 Tablets in the Treatment of Adult Patients With Moderate to Severe Plaque-type Psoriasis

Dermavon Holdings Limited — PHASE2, PHASE3

TrialNOT YET RECRUITING
Feb 2026Phase II Study of CMS-D002 Capsule for Uterine Fibroids With Menorrhagia

Shenzhen Kangzhe Biotechnology Co., Ltd. — PHASE2

TrialRECRUITING
Oct 2025Healthcare Integrity & Anti-Fraud Enforcement

Truway Health, Inc.

TrialENROLLING BY INVITATION
Jul 2025Erythrocytapheresis for Chronic Mountain Sickness

Third Military Medical University — NA

TrialRECRUITING
May 2025A Prospective Natural History and Outcome Measure Validation Study of Congenital Myasthenic Syndromes

National Institute of Neurological Disorders and Stroke (NINDS)

TrialRECRUITING
Mar 2025IPSC-CMs Combined with LVAD or CABG for the Treatment of Heart Failure

HELP Therapeutics Co., Ltd. — PHASE1, PHASE2

TrialNOT YET RECRUITING
Jan 2025Patients With Congenital Myasthenic Syndrome Will be Treated With Mesenchymal Stem Cell Exosome Solution

The Foundation for Orthopaedics and Regenerative Medicine — PHASE1

TrialRECRUITING
Sep 2024A Phase 1b Study to Investigate Safety and Tolerability of ARGX-119 in Adult Participants With DOK7-Congenital Myasthenic Syndromes (CMS)

argenx — PHASE1

TrialRECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Congenital myasthenic syndrome.

4 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

4 recruitingView all trials with filters →
Phase 12 trials
A Phase 1b Study to Investigate Safety and Tolerability of ARGX-119 in Adult Participants With DOK7-Congenital Myasthenic Syndromes (CMS)
Phase 1
Actively Recruiting
· Sites: Sacramento, California; Chicago, Illinois +7 more · Age: 1899 yrs
Patients With Congenital Myasthenic Syndrome Will be Treated With Mesenchymal Stem Cell Exosome Solution
Phase 1
Actively Recruiting
· Sites: Naples, Florida; Irving, Texas +1 more · Age: 1899 yrs
Other2 trials
A Prospective Natural History and Outcome Measure Validation Study of Congenital Myasthenic Syndromes
Actively Recruiting
PI: Christopher Grunseich, M.D. (National Institute of Neurological Disorders and S) · Sites: Bethesda, Maryland · Age: 099 yrs
A Natural History Study in Participants With Congenital Myasthenic Syndromes (CMS) Due to Mutations in DOK7, MUSK, AGRN, or LRP4
Actively Recruiting
· Sites: Sacramento, California; Chicago, Illinois +17 more · Age: 299 yrs

Specialists

7 foundView all specialists →
TM
Thomas Crawford, M.D.
Specialist
PI on 1 active trial
AM
Andrew G Engel, MD
Specialist
PI on 1 active trial1 Congenital myasthenic syndrome publication
LM
Laurent Servais, MD
Specialist
PI on 6 active trials1 Congenital myasthenic syndrome publication
CM
Christopher Grunseich, M.D.
Bethesda, Maryland
Specialist

Rare Disease Specialist

PI on 5 active trials
LP
Laurent SERVAIS, MD, PhD
Specialist
PI on 1 active trial
SE
Simon Edvardson
Specialist
PI on 1 active trial
VJ
Vern C. Juel
DURHAM, NC
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Congenital myasthenic syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Congenital myasthenic syndrome

Disease timeline:

New recruiting trial: The Role of the Tumor Molecular Profile (CMS), UGT1A1 Genotype and Beta-glucuronidase Activity of the Intestinal Microbiota for Treatment Efficiency, Toxicity, Survival and Quality of Life in Patients With Metastatic or Unresectable Colorectal Cancer During Irinotecan-based Systemic Treatment

A new clinical trial is recruiting patients for Congenital myasthenic syndrome

New recruiting trial: Phase II Study of CMS-D002 Capsule for Uterine Fibroids With Menorrhagia

A new clinical trial is recruiting patients for Congenital myasthenic syndrome

New recruiting trial: Erythrocytapheresis for Chronic Mountain Sickness

A new clinical trial is recruiting patients for Congenital myasthenic syndrome

New recruiting trial: Assessment of Allogeneic Hematopoietic Cell Transplantation in Medicare Beneficiaries With Multiple Myeloma

A new clinical trial is recruiting patients for Congenital myasthenic syndrome

New recruiting trial: Human Embryonic Stem Cell-Derived Cardiomyocyte Therapy for Chronic Ischemic Left Ventricular Dysfunction

A new clinical trial is recruiting patients for Congenital myasthenic syndrome

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

A new clinical trial is recruiting patients for Congenital myasthenic syndrome

New recruiting trial: A Prospective Natural History and Outcome Measure Validation Study of Congenital Myasthenic Syndromes

A new clinical trial is recruiting patients for Congenital myasthenic syndrome

New recruiting trial: Patients With Congenital Myasthenic Syndrome Will be Treated With Mesenchymal Stem Cell Exosome Solution

A new clinical trial is recruiting patients for Congenital myasthenic syndrome

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.Which gene is causing my CMS, and how does that affect my treatment options?,Which medications are safe for me, and which ones should I avoid?,Do I need breathing tests, and should I use any breathing support at night?,What signs should prompt me to go to the emergency room?,Are there clinical trials or new treatments I should know about?,Should other family members be tested for this condition?,What therapies or support services would help me manage daily life better?

Common questions about Congenital myasthenic syndrome

What is Congenital myasthenic syndrome?

Congenital myasthenic syndrome (CMS), sometimes called congenital myasthenia, is a group of rare inherited disorders that affect the way nerve signals are sent to muscles. In a healthy body, nerves and muscles communicate at a junction called the neuromuscular junction. In CMS, this communication is disrupted because of a genetic change, causing muscles to receive weak or unreliable signals. This leads to muscle weakness that is often present from birth or early childhood, though some forms appear later in life. The weakness in CMS can affect many parts of the body. The muscles around the eye

Are there clinical trials for Congenital myasthenic syndrome?

Yes — 4 recruiting clinical trials are currently listed for Congenital myasthenic syndrome on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Congenital myasthenic syndrome?

7 specialists and care centers treating Congenital myasthenic syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.