Distal myopathy, Welander type

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ORPHA:603OMIM:604454G71.0
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Overview

Distal myopathy, Welander type (also called Welander distal myopathy or WDM) is a rare inherited muscle disease that mainly affects the muscles farthest from the center of the body — the hands and feet. It was first described by Swedish neurologist Lisa Welander in 1951. The disease causes the small muscles in the fingers and hands to become weak first, making it hard to grip objects, button clothes, or do fine tasks with your hands. Over time, weakness can spread to the feet and lower legs, causing difficulty lifting the front of the foot (a problem called foot drop) and making walking harder. Welander distal myopathy is caused by a change (mutation) in a gene called TIA1. This gene helps control how cells handle stress and manage proteins. The disease progresses slowly over many years, and most people maintain the ability to walk well into older age. It does not typically affect the heart or breathing muscles. There is currently no cure for Welander distal myopathy. Treatment focuses on managing symptoms, maintaining strength and mobility through physical therapy, and using assistive devices like braces or splints when needed. Regular follow-up with a neuromuscular specialist is important to monitor how the disease is progressing and to adjust care over time.

Also known as:

Key symptoms:

Weakness in the fingers and handsDifficulty with fine hand movements like buttoning clothes or writingWeakness in the feet and lower legsFoot drop — difficulty lifting the front of the foot when walkingStumbling or tripping more than usualMuscle wasting (thinning) in the hands and lower legsReduced grip strengthDifficulty walking on uneven surfacesSlow, gradual worsening of muscle weakness over years

Clinical phenotype terms (13)— hover any for plain English
Intrinsic hand muscle atrophyHP:0008954Distal upper limb muscle weaknessHP:0008959Weakness of long finger extensor musclesHP:0009077Steppage gaitHP:0003376Distal upper limb amyotrophyHP:0007149
Inheritance

Autosomal dominant

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

Age of Onset

Late onset

Begins later in life, typically after age 50

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Distal myopathy, Welander type.

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No actively recruiting trials found for Distal myopathy, Welander type at this time.

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No specialists are currently listed for Distal myopathy, Welander type.

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 Distal myopathy, Welander type.

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Community

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Questions for your doctor

Bring these to your next appointment

  • Q1.What genetic test should I have to confirm the diagnosis, and how long will results take?,How quickly is my condition likely to progress, and what signs should I watch for?,Should other family members be tested for the TIA1 mutation?,What physical or occupational therapy would you recommend for me right now?,Are there any clinical trials or research studies I could participate in?,What assistive devices or braces might help me with my hand weakness or foot drop?,How often should I have follow-up appointments to monitor my muscle strength?

Common questions about Distal myopathy, Welander type

What is Distal myopathy, Welander type?

Distal myopathy, Welander type (also called Welander distal myopathy or WDM) is a rare inherited muscle disease that mainly affects the muscles farthest from the center of the body — the hands and feet. It was first described by Swedish neurologist Lisa Welander in 1951. The disease causes the small muscles in the fingers and hands to become weak first, making it hard to grip objects, button clothes, or do fine tasks with your hands. Over time, weakness can spread to the feet and lower legs, causing difficulty lifting the front of the foot (a problem called foot drop) and making walking harder

How is Distal myopathy, Welander type inherited?

Distal myopathy, Welander type follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Distal myopathy, Welander type typically begin?

Typical onset of Distal myopathy, Welander type is late onset. Age of onset can vary across affected individuals.