RERE-related neurodevelopmental syndrome

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ORPHA:494344OMIM:616975Q87.8
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Overview

RERE-related neurodevelopmental syndrome is a rare genetic condition caused by changes (variants) or deletions in the RERE gene. It is also sometimes called 'chromosome 1p36 deletion syndrome-like disorder' when a small piece of chromosome 1 containing the RERE gene is missing, though it can also occur from a change within the gene itself. This condition affects brain development and can also impact the eyes, heart, kidneys, and other organs. People with this syndrome typically show signs early in life, including intellectual disability, delayed speech and motor development, and behavioral challenges. Many also have features such as low muscle tone (hypotonia), seizures, and distinct facial features. Eye problems, hearing loss, and heart defects are also reported in some individuals. There is currently no cure for RERE-related neurodevelopmental syndrome. Treatment focuses on managing symptoms and supporting development. This includes early intervention therapies such as speech therapy, physical therapy, and occupational therapy. Seizures may be managed with anti-epileptic medications. A team of specialists is usually needed to address the many ways this condition can affect the body. With the right support, many individuals can make meaningful developmental progress.

Key symptoms:

Intellectual disability or learning difficultiesDelayed speech and language developmentDelayed motor milestones such as sitting or walking lateLow muscle tone (hypotonia)Seizures or epilepsyBehavioral challenges such as autism-like features or hyperactivityDistinct facial featuresEye problems such as vision loss or structural eye abnormalitiesHearing lossHeart defectsKidney or urinary tract abnormalitiesShort statureFeeding difficulties in infancy

Clinical phenotype terms (50)— hover any for plain English
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 RERE-related neurodevelopmental syndrome.

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No actively recruiting trials found for RERE-related neurodevelopmental syndrome at this time.

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No specialists are currently listed for RERE-related neurodevelopmental 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 RERE-related neurodevelopmental syndrome.

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Community

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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 change was found in the RERE gene, and what does that mean for my child's prognosis?,Should other family members be tested for this genetic change?,What therapies should we start right away, and how often should they happen?,What specialists does my child need to see, and how often?,What signs should prompt me to seek emergency care?,Are there any clinical trials or research studies we could participate in?,What support services or educational resources are available for my child?

Common questions about RERE-related neurodevelopmental syndrome

What is RERE-related neurodevelopmental syndrome?

RERE-related neurodevelopmental syndrome is a rare genetic condition caused by changes (variants) or deletions in the RERE gene. It is also sometimes called 'chromosome 1p36 deletion syndrome-like disorder' when a small piece of chromosome 1 containing the RERE gene is missing, though it can also occur from a change within the gene itself. This condition affects brain development and can also impact the eyes, heart, kidneys, and other organs. People with this syndrome typically show signs early in life, including intellectual disability, delayed speech and motor development, and behavioral ch

How is RERE-related neurodevelopmental syndrome inherited?

RERE-related neurodevelopmental syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does RERE-related neurodevelopmental syndrome typically begin?

Typical onset of RERE-related neurodevelopmental syndrome is infantile. Age of onset can vary across affected individuals.