Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:439212OMIM:614399G71.2
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

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

Overview

Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome (sometimes called EMARDD) is a very rare genetic muscle disease that begins in infancy or early childhood. It is caused by mutations in the MEGF10 gene, which plays an important role in muscle development and repair. Children with this condition are born with or quickly develop significant muscle weakness (myopathy), absent reflexes (areflexia), difficulty breathing (respiratory distress), and trouble swallowing (dysphagia). The muscle weakness tends to be widespread and can be severe, often requiring breathing support early in life. Because the muscles used for breathing and swallowing are affected, children may need a ventilator or feeding tube. The disease can also affect the ability to move and reach normal developmental milestones like sitting or walking. Currently, there is no cure for EMARDD. Treatment focuses on managing symptoms and supporting breathing, nutrition, and mobility. A team of specialists including neurologists, pulmonologists, and geneticists typically provides care. Research into this condition is ongoing, but because it is extremely rare, knowledge about the full range of outcomes is still growing.

Also known as:

Key symptoms:

Severe muscle weakness from birth or early infancyAbsent reflexes (areflexia)Difficulty breathing or respiratory failureDifficulty swallowingPoor muscle tone (floppy baby)Delayed motor milestones such as sitting or walkingFeeding difficultiesNeed for ventilator supportMuscle wastingScoliosis or spinal curvatureFrequent respiratory infectionsWeak cryLimited limb movement

Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome.

View clinical trials →

No actively recruiting trials found for Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome community →

No specialists are currently listed for Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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 Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Early-onset myopathy-areflexia-respiratory distress-dysphagia syndromeForum →

No community posts yet. Be the first to share your experience with Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome.

Start the conversation →

Latest news about Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome

No recent news articles for Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome.

Follow this condition to be notified when news becomes available.

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.How severe is my child's muscle weakness, and what level of breathing support will they need?,Should we pursue genetic testing to confirm the diagnosis, and should other family members be tested?,What is the best feeding plan to prevent aspiration and ensure proper nutrition?,What therapies (physical, occupational, speech) should we start, and how often?,Are there any clinical trials or research studies we could participate in?,What emergency signs should we watch for at home, and what is the emergency plan?,How can we connect with other families affected by this condition?

Common questions about Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome

What is Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome?

Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome (sometimes called EMARDD) is a very rare genetic muscle disease that begins in infancy or early childhood. It is caused by mutations in the MEGF10 gene, which plays an important role in muscle development and repair. Children with this condition are born with or quickly develop significant muscle weakness (myopathy), absent reflexes (areflexia), difficulty breathing (respiratory distress), and trouble swallowing (dysphagia). The muscle weakness tends to be widespread and can be severe, often requiring breathing support earl

How is Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome inherited?

Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome typically begin?

Typical onset of Early-onset myopathy-areflexia-respiratory distress-dysphagia syndrome is neonatal. Age of onset can vary across affected individuals.