Gabriele-de Vries syndrome

Last reviewed

🖨 Print for my doctorAdvocacy Hub →
ORPHA:506358OMIM:617557Q87.8
Who is this for?
Show terms as
8Treatment centers

Where are you in your journey?

UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
Report missing data

Overview

Gabriele-de Vries syndrome (also sometimes called YWHAE-related intellectual disability or YDvS) is a rare genetic condition caused by changes in a gene called YWHAE. This gene plays an important role in how brain cells develop and communicate with each other. The syndrome mainly affects brain development, leading to intellectual disability, delayed speech and language, and behavioral challenges. Most people with this condition also have distinctive facial features that doctors can recognize. The condition affects multiple areas of development. Children with Gabriele-de Vries syndrome often have delays in reaching milestones like talking and learning. Many also experience behavioral issues such as attention problems, anxiety, or features similar to autism spectrum disorder. Some individuals have mild differences in muscle tone, making movement slightly harder in early childhood. Seizures have also been reported in some affected individuals. Currently, there is no cure for Gabriele-de Vries syndrome. Treatment focuses on managing symptoms and supporting development. This includes speech therapy, occupational therapy, physical therapy, special education support, and medications to manage seizures or behavioral symptoms when needed. With the right support, many individuals can make meaningful progress in communication and daily skills.

Also known as:

Key symptoms:

Intellectual disability (ranging from mild to moderate)Delayed speech and language developmentBehavioral challenges such as anxiety, attention difficulties, or autism-like featuresDistinctive facial features recognized by specialistsLow muscle tone (hypotonia) in infancyDelayed walking or motor milestonesSeizures in some individualsFeeding difficulties in infancyShort stature in some casesSleep disturbances

Clinical phenotype terms (50)— hover any for plain English
Periorbital fullnessHP:0000629Abnormality of upper lip vermillionHP:0011339
Inheritance

Autosomal dominant

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

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Gabriele-de Vries syndrome.

View clinical trials →

No actively recruiting trials found for Gabriele-de Vries syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Gabriele-de Vries syndrome community →

No specialists are currently listed for Gabriele-de Vries 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 Gabriele-de Vries syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

Open Gabriele-de Vries syndromeForum →

No community posts yet. Be the first to share your experience with Gabriele-de Vries syndrome.

Start the conversation →

Latest news about Gabriele-de Vries syndrome

No recent news articles for Gabriele-de Vries syndrome.

Follow this condition to be notified when news becomes available.

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 type of genetic change does my child have in the YWHAE gene, and what does that mean for their prognosis?,Should other family members be tested for this genetic change?,What therapies do you recommend starting first, and how soon?,What signs of seizures should I watch for, and what should I do if one happens?,Are there any clinical trials or research studies we could participate in?,What kind of educational support will my child need, and how do I advocate for an IEP?,What does long-term care look like for adults with this condition?

Common questions about Gabriele-de Vries syndrome

What is Gabriele-de Vries syndrome?

Gabriele-de Vries syndrome (also sometimes called YWHAE-related intellectual disability or YDvS) is a rare genetic condition caused by changes in a gene called YWHAE. This gene plays an important role in how brain cells develop and communicate with each other. The syndrome mainly affects brain development, leading to intellectual disability, delayed speech and language, and behavioral challenges. Most people with this condition also have distinctive facial features that doctors can recognize. The condition affects multiple areas of development. Children with Gabriele-de Vries syndrome often h

How is Gabriele-de Vries syndrome inherited?

Gabriele-de Vries syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Gabriele-de Vries syndrome typically begin?

Typical onset of Gabriele-de Vries syndrome is infantile. Age of onset can vary across affected individuals.