Scapuloperoneal spinal muscular atrophy

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ORPHA:431255OMIM:181405G12.1
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8Treatment centers

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Overview

Scapuloperoneal spinal muscular atrophy (also called SPSMA or scapuloperoneal SMA) is a rare inherited nerve and muscle disease. It belongs to a group of conditions called spinal muscular atrophies, where the nerve cells in the spinal cord that control muscle movement slowly break down over time. The name describes where weakness tends to show up first: the shoulder blade area (scapular) and the lower legs and feet (peroneal). This pattern of weakness is one of the most recognizable features of the disease. People with this condition usually notice weakness and wasting of the muscles around the shoulder blades, making it hard to lift the arms above the head. At the same time, or sometimes later, the muscles in the lower legs weaken, which can cause foot drop — a condition where the foot hangs down and makes walking difficult. Some people also develop problems with their voice or swallowing if the nerves controlling those muscles are affected. There is currently no cure for scapuloperoneal spinal muscular atrophy. Treatment focuses on managing symptoms, maintaining mobility, and improving quality of life. Physical therapy, occupational therapy, and assistive devices like ankle-foot braces are commonly used. A team of specialists works together to help people stay as active and independent as possible. The disease tends to progress slowly in most people, and many individuals live a normal or near-normal lifespan.

Also known as:

Key symptoms:

Weakness and wasting of muscles around the shoulder bladesDifficulty lifting arms above the headWeakness in the lower legs and feetFoot drop (the foot hangs down, making it hard to walk normally)Winging of the shoulder blades (they stick out from the back)Muscle wasting in the calves and lower legsDifficulty walking or frequent tripping and fallingWeakness that slowly gets worse over timePossible voice changes or hoarsenessPossible difficulty swallowing in some casesReduced reflexes in affected limbs

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Scapuloperoneal spinal muscular atrophy.

View clinical trials →

No actively recruiting trials found for Scapuloperoneal spinal muscular atrophy at this time.

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

Search ClinicalTrials.gov ↗Join the Scapuloperoneal spinal muscular atrophy community →

No specialists are currently listed for Scapuloperoneal spinal muscular atrophy.

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 Scapuloperoneal spinal muscular atrophy.

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Community

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Latest news about Scapuloperoneal spinal muscular atrophy

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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.Which gene is causing my condition, and what does that mean for my family members?,How quickly do you expect my weakness to progress, based on my current exam and test results?,What therapies or braces would help me most right now?,Should I have my breathing tested, and how often should it be monitored?,Are there any clinical trials I might be eligible for?,What warning signs should prompt me to seek urgent medical care?,Are there patient registries or support groups I should know about?

Common questions about Scapuloperoneal spinal muscular atrophy

What is Scapuloperoneal spinal muscular atrophy?

Scapuloperoneal spinal muscular atrophy (also called SPSMA or scapuloperoneal SMA) is a rare inherited nerve and muscle disease. It belongs to a group of conditions called spinal muscular atrophies, where the nerve cells in the spinal cord that control muscle movement slowly break down over time. The name describes where weakness tends to show up first: the shoulder blade area (scapular) and the lower legs and feet (peroneal). This pattern of weakness is one of the most recognizable features of the disease. People with this condition usually notice weakness and wasting of the muscles around t

How is Scapuloperoneal spinal muscular atrophy inherited?

Scapuloperoneal spinal muscular atrophy follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.