Intellectual disability-obesity-brain malformations-facial dysmorphism syndrome

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ORPHA:352530OMIM:613192Q04.8
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Overview

Intellectual disability-obesity-brain malformations-facial dysmorphism syndrome is an extremely rare genetic condition that affects brain development, body weight, facial features, and intellectual ability. This syndrome is sometimes referred to by its Orphanet designation (ORPHA:352530) and is caused by genetic changes that disrupt normal development before and after birth. Children born with this condition typically show intellectual disability ranging from mild to severe, meaning they may have difficulty learning, reasoning, and developing skills at the expected pace. Obesity often develops in early childhood and can be difficult to manage. Brain imaging (such as MRI) may reveal structural abnormalities in the brain, including malformations of the cerebral cortex or other brain regions. Affected individuals also tend to have distinctive facial features, which may include a broad forehead, widely spaced eyes, a flat nasal bridge, or other subtle differences. Because this syndrome is so rare, there is no specific cure or targeted treatment available. Management focuses on supportive care, including special education services, speech and occupational therapy, dietary management for obesity, and monitoring for any associated medical complications. A team of specialists typically works together to address the various needs of affected individuals and their families.

Also known as:

Key symptoms:

Intellectual disabilityObesity beginning in childhoodBrain structure abnormalities seen on imagingDistinctive facial featuresDelayed speech and language developmentDelayed motor milestones such as sitting and walkingLearning difficultiesBehavioral challengesLow muscle tone in infancyBroad or flat facial appearanceWidely spaced eyesSeizures in some casesShort stature in some individuals

Clinical phenotype terms (31)— hover any for plain English
Congenital hypothyroidismHP:0000851Malignant hyperthermiaHP:0002047
Inheritance

Autosomal recessive

Passed on when both parents carry the same gene change; often skips generations

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 Intellectual disability-obesity-brain malformations-facial dysmorphism syndrome.

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No actively recruiting trials found for Intellectual disability-obesity-brain malformations-facial dysmorphism syndrome at this time.

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No specialists are currently listed for Intellectual disability-obesity-brain malformations-facial dysmorphism 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 Intellectual disability-obesity-brain malformations-facial dysmorphism 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

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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 genetic change was found, and what does it mean for my child's health?,What therapies should we start right away to support my child's development?,How should we manage my child's weight, and should we see a dietitian?,Are there any brain abnormalities we should monitor over time with repeat imaging?,Is my child at risk for seizures, and what should I watch for?,What is the chance of having another child with the same condition?,Are there any clinical trials or research studies we could participate in?

Common questions about Intellectual disability-obesity-brain malformations-facial dysmorphism syndrome

What is Intellectual disability-obesity-brain malformations-facial dysmorphism syndrome?

Intellectual disability-obesity-brain malformations-facial dysmorphism syndrome is an extremely rare genetic condition that affects brain development, body weight, facial features, and intellectual ability. This syndrome is sometimes referred to by its Orphanet designation (ORPHA:352530) and is caused by genetic changes that disrupt normal development before and after birth. Children born with this condition typically show intellectual disability ranging from mild to severe, meaning they may have difficulty learning, reasoning, and developing skills at the expected pace. Obesity often develop

How is Intellectual disability-obesity-brain malformations-facial dysmorphism syndrome inherited?

Intellectual disability-obesity-brain malformations-facial dysmorphism syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Intellectual disability-obesity-brain malformations-facial dysmorphism syndrome typically begin?

Typical onset of Intellectual disability-obesity-brain malformations-facial dysmorphism syndrome is infantile. Age of onset can vary across affected individuals.