Ullrich congenital muscular dystrophy

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ORPHA:75840OMIM:620727G71.2
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1Specialists8Treatment centers

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Overview

Ullrich congenital muscular dystrophy (UCMD) is a rare inherited muscle disease that is present from birth or becomes noticeable in the first months of life. It belongs to a group of conditions called congenital muscular dystrophies, which means the muscles are affected from very early on. UCMD is caused by problems in proteins called collagens (specifically collagen VI), which normally help hold muscle fibers together and keep them healthy. Without enough working collagen VI, muscle cells become damaged over time and cannot repair themselves properly. Children with UCMD typically have weak muscles throughout the body, which makes it hard to move, sit, stand, or walk. One of the most distinctive features is a combination of very stiff joints in some areas (like the spine and hips) alongside unusually flexible joints in others (like the fingers, wrists, and ankles). Breathing muscles can also weaken over time, which is one of the most serious concerns in this condition. There is currently no cure for UCMD. Treatment focuses on managing symptoms, supporting breathing, maintaining mobility, and improving quality of life. A team of specialists including neurologists, pulmonologists, and physical therapists work together to help patients live as comfortably and independently as possible. Research into gene therapy and other targeted treatments is ongoing, giving hope for future options.

Also known as:

Key symptoms:

Muscle weakness from birth or early infancyDifficulty sitting, standing, or walkingStiff spine and hips (reduced flexibility)Unusually flexible fingers, wrists, and ankles (hypermobility)Breathing difficulties, especially during sleepCurved spine (scoliosis)Protruding heels (calcanei)Soft, doughy skin textureFeeding difficulties in infancyDelayed motor milestones (sitting, walking)Fatigue with minimal physical activityContractures (joints that become permanently bent or stiff over time)Respiratory failure in severe cases

Clinical phenotype terms (31)— hover any for plain English
Spinal rigidityHP:0003306Abnormal muscle fiber morphologyHP:0004303Wrist hypermobilityHP:0005072Increased laxity of fingersHP:0006149Increased endomysial connective tissueHP:0100297Slender fingerHP:0001238
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 Ullrich congenital muscular dystrophy.

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No actively recruiting trials found for Ullrich congenital muscular dystrophy at this time.

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Specialists

1 foundView all specialists →

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 Ullrich congenital muscular dystrophy.

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Community

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Latest news about Ullrich congenital muscular dystrophy

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Caregiver Resources

NORD Caregiver Resources

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

Mental Health Support

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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 genes should be tested, and what does the result mean for my child and our family?,When should we start monitoring breathing, and what signs should prompt us to seek help immediately?,What physical therapy program is best for my child's current level of function?,Are there any clinical trials or research studies we should consider enrolling in?,How often should we see each specialist, and who should coordinate overall care?,What assistive devices or home modifications will help my child be as independent as possible?,What is the likelihood that other family members or future children could be affected?

Common questions about Ullrich congenital muscular dystrophy

What is Ullrich congenital muscular dystrophy?

Ullrich congenital muscular dystrophy (UCMD) is a rare inherited muscle disease that is present from birth or becomes noticeable in the first months of life. It belongs to a group of conditions called congenital muscular dystrophies, which means the muscles are affected from very early on. UCMD is caused by problems in proteins called collagens (specifically collagen VI), which normally help hold muscle fibers together and keep them healthy. Without enough working collagen VI, muscle cells become damaged over time and cannot repair themselves properly. Children with UCMD typically have weak m

How is Ullrich congenital muscular dystrophy inherited?

Ullrich congenital muscular dystrophy follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Ullrich congenital muscular dystrophy typically begin?

Typical onset of Ullrich congenital muscular dystrophy is neonatal. Age of onset can vary across affected individuals.

Which specialists treat Ullrich congenital muscular dystrophy?

1 specialists and care centers treating Ullrich congenital muscular dystrophy are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.