Developmental delay-facial dysmorphism syndrome due to MED13L deficiency

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ORPHA:369891OMIM:616789Q87.0
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Overview

Developmental delay-facial dysmorphism syndrome due to MED13L deficiency, also known as MED13L syndrome or MED13L-related intellectual disability, is a rare genetic condition caused by changes (mutations) in the MED13L gene. This gene provides instructions for making a protein that is part of a large complex called the Mediator complex, which helps control how other genes are turned on and off during development. When the MED13L gene does not work properly, it can affect brain development and other parts of the body. The main features of this condition include developmental delay, intellectual disability (ranging from mild to severe), speech and language difficulties, and distinctive facial features. Many children with this syndrome have low muscle tone (hypotonia), delayed motor milestones such as sitting and walking, and significant challenges with speech development. Some individuals may also experience seizures, behavioral differences, and problems with coordination. There is currently no cure for MED13L syndrome. Treatment focuses on managing symptoms and supporting development through therapies such as speech therapy, physical therapy, occupational therapy, and special education services. Early intervention programs can make a meaningful difference in helping children reach their full potential. Some individuals may also need medications to manage seizures or behavioral challenges. With appropriate support, many people with MED13L syndrome can make steady developmental progress over time.

Also known as:

Key symptoms:

Developmental delayIntellectual disabilityDelayed or absent speechLow muscle tone (floppiness)Delayed walkingDistinctive facial featuresSeizures or epilepsyWide or flat nasal bridgeOpen mouth appearanceBehavioral challenges such as autism-like featuresPoor coordination or clumsinessFeeding difficulties in infancyShort attention spanFriendly or happy demeanorAbnormal brain findings on MRI

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 Developmental delay-facial dysmorphism syndrome due to MED13L deficiency.

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No actively recruiting trials found for Developmental delay-facial dysmorphism syndrome due to MED13L deficiency at this time.

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No specialists are currently listed for Developmental delay-facial dysmorphism syndrome due to MED13L deficiency.

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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

No travel grants are currently matched to Developmental delay-facial dysmorphism syndrome due to MED13L deficiency.

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Community

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Caregiver Resources

NORD Caregiver Resources

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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 type of MED13L mutation does my child have, and does it affect the expected severity?,What therapies should we start right away, and how often should they occur?,Should my child have a brain MRI or EEG to check for seizures or brain differences?,Are there any clinical trials or research studies we should know about?,What educational supports and accommodations should we request at school?,How likely is it that my child will develop speech, and what communication tools can help in the meantime?,Is there a risk of this condition occurring again in future pregnancies?

Common questions about Developmental delay-facial dysmorphism syndrome due to MED13L deficiency

What is Developmental delay-facial dysmorphism syndrome due to MED13L deficiency?

Developmental delay-facial dysmorphism syndrome due to MED13L deficiency, also known as MED13L syndrome or MED13L-related intellectual disability, is a rare genetic condition caused by changes (mutations) in the MED13L gene. This gene provides instructions for making a protein that is part of a large complex called the Mediator complex, which helps control how other genes are turned on and off during development. When the MED13L gene does not work properly, it can affect brain development and other parts of the body. The main features of this condition include developmental delay, intellectua

How is Developmental delay-facial dysmorphism syndrome due to MED13L deficiency inherited?

Developmental delay-facial dysmorphism syndrome due to MED13L deficiency follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Developmental delay-facial dysmorphism syndrome due to MED13L deficiency typically begin?

Typical onset of Developmental delay-facial dysmorphism syndrome due to MED13L deficiency is infantile. Age of onset can vary across affected individuals.