Emery-Nelson syndrome

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ORPHA:1927OMIM:139750Q87.8
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Overview

Emery-Nelson syndrome is an extremely rare genetic condition that primarily affects males and is characterized by a combination of intellectual disability, short stature, and distinctive physical features. The syndrome was first described in the medical literature and has been reported in only a very small number of families worldwide. Affected individuals typically show mild to moderate intellectual disability, which may become apparent during early childhood when developmental milestones such as speech and learning are delayed. Physical features can include a short neck, broad chest, and certain facial characteristics. Some individuals may also experience joint limitations or mild skeletal abnormalities. Because this condition is so rare, the full range of symptoms and their severity can vary between affected individuals. There is currently no cure for Emery-Nelson syndrome. Treatment focuses on managing individual symptoms and supporting development through therapies such as speech therapy, occupational therapy, and special education services. Regular follow-up with a team of specialists is important to monitor growth, development, and any emerging health concerns over time.

Also known as:

Key symptoms:

Intellectual disabilityShort statureDelayed speech developmentDelayed motor milestonesShort neckBroad chestJoint stiffness or limited joint movementDistinctive facial featuresLearning difficultiesMild skeletal abnormalities

Clinical phenotype terms (12)— hover any for plain English
Metacarpophalangeal joint contractureHP:0006070Interphalangeal thumb joint contractureHP:0009626
Inheritance

X-linked recessive

Carried on the X chromosome; typically affects males more than females

Age of Onset

Childhood

Begins in childhood, roughly ages 1 to 12

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Emery-Nelson syndrome.

View clinical trials →

No actively recruiting trials found for Emery-Nelson syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Emery-Nelson syndrome community →

No specialists are currently listed for Emery-Nelson syndrome.

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 Emery-Nelson syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Emery-Nelson syndrome

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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.What specific developmental therapies would benefit my child the most right now?,Is genetic testing available that could confirm the diagnosis or identify the specific gene involved?,What should I watch for as my child grows that might need medical attention?,Are there any clinical studies or research programs we could participate in?,What educational supports and accommodations should we request at school?,Should female family members be tested to see if they are carriers?,How often should we schedule follow-up visits with specialists?

Common questions about Emery-Nelson syndrome

What is Emery-Nelson syndrome?

Emery-Nelson syndrome is an extremely rare genetic condition that primarily affects males and is characterized by a combination of intellectual disability, short stature, and distinctive physical features. The syndrome was first described in the medical literature and has been reported in only a very small number of families worldwide. Affected individuals typically show mild to moderate intellectual disability, which may become apparent during early childhood when developmental milestones such as speech and learning are delayed. Physical features can include a short neck, broad chest, and cer

How is Emery-Nelson syndrome inherited?

Emery-Nelson syndrome follows a x-linked recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Emery-Nelson syndrome typically begin?

Typical onset of Emery-Nelson syndrome is childhood. Age of onset can vary across affected individuals.