Overview
Dejerine-Sottas syndrome (DSS) is a rare and severe inherited nerve disease that affects the peripheral nervous system — the network of nerves outside the brain and spinal cord. It is sometimes called Dejerine-Sottas disease, hereditary motor and sensory neuropathy type III (HMSN III), or DSS. The condition causes the protective covering around nerve fibers (called myelin) to break down, which slows or blocks the signals nerves send to muscles and skin. This leads to significant muscle weakness, loss of coordination, and reduced ability to feel touch, pain, or temperature. Symptoms usually appear in infancy or early childhood and tend to be more severe than related nerve diseases like Charcot-Marie-Tooth disease. Children with DSS may be slow to reach motor milestones like sitting, standing, or walking. Over time, many people develop foot deformities, scoliosis (curved spine), and may lose the ability to walk independently. Breathing can also be affected in some cases. There is currently no cure for Dejerine-Sottas syndrome. Treatment focuses on managing symptoms, maintaining mobility, and improving quality of life through physical therapy, occupational therapy, orthopedic devices, and sometimes surgery. A team of specialists works together to support people living with this condition throughout their lives.
Also known as:
Key symptoms:
Severe muscle weakness, especially in the arms and legsLoss of feeling or numbness in the hands and feetDifficulty walking or loss of ability to walkPoor balance and coordinationFoot deformities such as high arches or flat feetCurved spine (scoliosis)Enlarged, thickened nerves that can sometimes be felt under the skinReduced or absent reflexes (for example, no knee-jerk reflex)Tremors or shakingDelayed motor milestones in infancy and childhoodBreathing difficulties in severe casesFatigue due to the effort of moving weakened musclesSensitivity to touch or abnormal sensations like tingling
Variable
Can be inherited in different ways depending on the underlying gene
Infantile
Begins in infancy, roughly 1 month to 2 years old
Treatments
No FDA-approved treatments are currently listed for Dejerine-Sottas syndrome.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Dejerine-Sottas syndrome at this time.
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Dejerine-Sottas syndrome.
Community
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Start the conversation →Latest news about Dejerine-Sottas syndrome
Disease timeline:
New recruiting trial: Effect of Night-time and Full-time Splinting for Carpal Tunnel Syndrome
A new clinical trial is recruiting patients for Dejerine-Sottas syndrome
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 mutation is causing my (or my child's) condition, and what does that mean for how the disease will progress?,What therapies or interventions should we start right away to protect muscle function and mobility?,How often should we have follow-up appointments, and which specialists should be part of our care team?,Are there any clinical trials or research studies we might be eligible for?,What signs should prompt us to seek emergency care?,Should other family members be tested for the same genetic mutation?,What school or workplace accommodations would you recommend, and can you help us document them?
Common questions about Dejerine-Sottas syndrome
What is Dejerine-Sottas syndrome?
Dejerine-Sottas syndrome (DSS) is a rare and severe inherited nerve disease that affects the peripheral nervous system — the network of nerves outside the brain and spinal cord. It is sometimes called Dejerine-Sottas disease, hereditary motor and sensory neuropathy type III (HMSN III), or DSS. The condition causes the protective covering around nerve fibers (called myelin) to break down, which slows or blocks the signals nerves send to muscles and skin. This leads to significant muscle weakness, loss of coordination, and reduced ability to feel touch, pain, or temperature. Symptoms usually ap
At what age does Dejerine-Sottas syndrome typically begin?
Typical onset of Dejerine-Sottas syndrome is infantile. Age of onset can vary across affected individuals.
Which specialists treat Dejerine-Sottas syndrome?
23 specialists and care centers treating Dejerine-Sottas syndrome are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.