Myopathic Ehlers-Danlos syndrome

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ORPHA:536516OMIM:616471Q79.6
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Overview

Myopathic Ehlers-Danlos syndrome (mEDS) is a rare inherited connective tissue disorder that primarily affects the muscles and joints. Unlike other forms of Ehlers-Danlos syndrome (EDS), which mainly cause very flexible joints and stretchy skin, mEDS is unique because it causes significant muscle weakness and wasting (loss of muscle mass) alongside joint problems. The condition is caused by changes (mutations) in the COL12A1 gene, which provides instructions for making a protein called collagen type XII. This protein plays an important role in keeping muscles and their surrounding tissues strong and organized. People with mEDS often experience muscle weakness from birth or early childhood, which can make it hard to reach normal movement milestones like sitting up, standing, or walking. Joints tend to be very loose and flexible (hypermobile), and the skin may be soft or mildly stretchy. Some people also develop a curved spine (scoliosis) or foot deformities over time. There is currently no cure for mEDS. Treatment focuses on managing symptoms, improving strength and function through physical therapy, and preventing complications like joint injuries or spinal problems. With the right support team and therapies, many people with mEDS can lead meaningful and active lives, though the level of disability varies from person to person.

Also known as:

Key symptoms:

Muscle weakness, especially in the arms and legsLow muscle tone (floppy muscles) from birthVery flexible or loose joints (joint hypermobility)Delayed motor milestones such as sitting, standing, or walkingMuscle wasting (loss of muscle bulk over time)Soft or mildly stretchy skinCurved spine (scoliosis)Foot deformities such as flat feet or clubfootEasy bruisingFatigue and low staminaDifficulty with activities requiring strength or endurance

Clinical phenotype terms (49)— hover any for plain English
Delayed ability to standHP:0025335Soft skinHP:0000977
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 Myopathic Ehlers-Danlos syndrome.

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No actively recruiting trials found for Myopathic Ehlers-Danlos syndrome at this time.

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No specialists are currently listed for Myopathic Ehlers-Danlos 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 Myopathic Ehlers-Danlos syndrome.

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Community

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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.What type of genetic testing do you recommend to confirm the diagnosis, and should other family members be tested?,How often should my child (or I) be seen by a physical therapist, and what exercises are safe to do at home?,Are there any activities or sports that should be avoided to protect the joints and muscles?,What signs of complications, such as scoliosis or breathing problems, should I watch for and report immediately?,Are there any clinical trials or research studies for mEDS that we might be eligible to join?,What specialists should be part of our care team, and how often should we see each one?,What support resources or patient organizations are available for families affected by mEDS?

Common questions about Myopathic Ehlers-Danlos syndrome

What is Myopathic Ehlers-Danlos syndrome?

Myopathic Ehlers-Danlos syndrome (mEDS) is a rare inherited connective tissue disorder that primarily affects the muscles and joints. Unlike other forms of Ehlers-Danlos syndrome (EDS), which mainly cause very flexible joints and stretchy skin, mEDS is unique because it causes significant muscle weakness and wasting (loss of muscle mass) alongside joint problems. The condition is caused by changes (mutations) in the COL12A1 gene, which provides instructions for making a protein called collagen type XII. This protein plays an important role in keeping muscles and their surrounding tissues stron

How is Myopathic Ehlers-Danlos syndrome inherited?

Myopathic Ehlers-Danlos syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Myopathic Ehlers-Danlos syndrome typically begin?

Typical onset of Myopathic Ehlers-Danlos syndrome is neonatal. Age of onset can vary across affected individuals.