Hereditary sensorimotor neuropathy with hyperelastic skin

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ORPHA:280598OMIM:608895G60.0
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Overview

Hereditary sensorimotor neuropathy with hyperelastic skin is an extremely rare genetic condition that combines two main problems: nerve damage affecting sensation and movement (sensorimotor neuropathy) and unusually stretchy, elastic skin. The nerve damage part of this condition is similar to what is seen in Charcot-Marie-Tooth disease (CMT), which is the most common inherited nerve disorder. Patients typically experience progressive weakness and loss of feeling in the hands and feet, along with skin that stretches more than normal and may bruise easily. The skin features overlap with those seen in Ehlers-Danlos syndrome. Symptoms usually begin in childhood or adolescence and tend to worsen slowly over time. The nerve damage can lead to difficulty walking, foot deformities such as high arches or hammertoes, and problems with fine motor skills in the hands. The hyperelastic skin may also be fragile and heal poorly after injuries. Some patients may have joint hypermobility, meaning their joints bend more than usual. There is currently no cure for this condition. Treatment focuses on managing symptoms and maintaining quality of life. Physical therapy, occupational therapy, braces or orthotics for the feet and ankles, and pain management are the main approaches. Skin care is also important to prevent injuries and promote healing. Regular monitoring by a team of specialists helps address the various aspects of this complex condition.

Key symptoms:

Progressive weakness in the feet and legsProgressive weakness in the handsNumbness or reduced sensation in the hands and feetUnusually stretchy or elastic skinSkin that bruises easilyPoor wound healingHigh foot arches (pes cavus)Hammertoes or other foot deformitiesDifficulty walking or frequent trippingJoint hypermobility or loose jointsMuscle wasting in the lower legsTingling or burning sensations in the extremitiesReduced reflexesDifficulty with fine motor tasks like buttoning clothes

Inheritance

Autosomal dominant

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

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Hereditary sensorimotor neuropathy with hyperelastic skin.

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No specialists are currently listed for Hereditary sensorimotor neuropathy with hyperelastic skin.

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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

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Community

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific genetic testing should be done to confirm the diagnosis?,How quickly is this condition likely to progress in my case?,What medications should I avoid because they could worsen nerve damage?,What physical therapy program would be most helpful?,Are there any clinical trials or research studies I could participate in?,How should I care for my skin to prevent injuries and promote healing?,What signs should prompt me to seek urgent medical attention?

Common questions about Hereditary sensorimotor neuropathy with hyperelastic skin

What is Hereditary sensorimotor neuropathy with hyperelastic skin?

Hereditary sensorimotor neuropathy with hyperelastic skin is an extremely rare genetic condition that combines two main problems: nerve damage affecting sensation and movement (sensorimotor neuropathy) and unusually stretchy, elastic skin. The nerve damage part of this condition is similar to what is seen in Charcot-Marie-Tooth disease (CMT), which is the most common inherited nerve disorder. Patients typically experience progressive weakness and loss of feeling in the hands and feet, along with skin that stretches more than normal and may bruise easily. The skin features overlap with those se

How is Hereditary sensorimotor neuropathy with hyperelastic skin inherited?

Hereditary sensorimotor neuropathy with hyperelastic skin follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Hereditary sensorimotor neuropathy with hyperelastic skin typically begin?

Typical onset of Hereditary sensorimotor neuropathy with hyperelastic skin is childhood. Age of onset can vary across affected individuals.