Corpus callosum agenesis-macrocephaly-hypertelorism syndrome

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Overview

Corpus callosum agenesis-macrocephaly-hypertelorism syndrome is an extremely rare genetic condition that affects brain development and facial features. The corpus callosum is a thick band of nerve fibers that connects the left and right halves of the brain, allowing them to communicate with each other. In this syndrome, the corpus callosum fails to develop (agenesis), which can affect how the brain processes information. Along with this brain difference, affected individuals typically have an unusually large head size (macrocephaly) and widely spaced eyes (hypertelorism). These features are usually noticed at birth or during early infancy. Children with this condition may experience developmental delays, intellectual disability, and difficulties with coordination and motor skills. The degree of these challenges can vary from person to person. Some individuals may also have seizures, speech and language delays, and behavioral differences. Facial features beyond widely spaced eyes may include a broad forehead and other distinctive characteristics. There is currently no cure for this syndrome. Treatment focuses on managing symptoms and supporting development. This may include physical therapy, occupational therapy, speech therapy, and special education services. Seizures, if present, are managed with anti-seizure medications. Early intervention programs can help children reach their full potential. A team of specialists typically works together to provide comprehensive care.

Also known as:

Key symptoms:

Missing or underdeveloped connection between brain halvesUnusually large head sizeWidely spaced eyesDevelopmental delaysIntellectual disabilitySpeech and language delaysSeizuresDifficulty with coordination and motor skillsBroad foreheadDistinctive facial featuresLearning difficultiesBehavioral challengesLow muscle tone

Clinical phenotype terms (10)— hover any for plain English
Chiari type I malformationHP:0007099Borderline intellectual disabilityHP:0006889
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Corpus callosum agenesis-macrocephaly-hypertelorism syndrome.

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No actively recruiting trials found for Corpus callosum agenesis-macrocephaly-hypertelorism syndrome at this time.

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No specialists are currently listed for Corpus callosum agenesis-macrocephaly-hypertelorism 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 Corpus callosum agenesis-macrocephaly-hypertelorism 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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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 therapies does my child need right now, and how often should they receive them?,Should we pursue genetic testing, and what type of testing do you recommend?,What is the risk of seizures, and what should I do if one occurs?,How will this condition affect my child's learning, and what educational supports should we request?,Are there any clinical trials or research studies that my child might be eligible for?,What specialists should be part of my child's care team, and how often should we see them?,What is the chance that future children could also be affected by this condition?

Common questions about Corpus callosum agenesis-macrocephaly-hypertelorism syndrome

What is Corpus callosum agenesis-macrocephaly-hypertelorism syndrome?

Corpus callosum agenesis-macrocephaly-hypertelorism syndrome is an extremely rare genetic condition that affects brain development and facial features. The corpus callosum is a thick band of nerve fibers that connects the left and right halves of the brain, allowing them to communicate with each other. In this syndrome, the corpus callosum fails to develop (agenesis), which can affect how the brain processes information. Along with this brain difference, affected individuals typically have an unusually large head size (macrocephaly) and widely spaced eyes (hypertelorism). These features are us

At what age does Corpus callosum agenesis-macrocephaly-hypertelorism syndrome typically begin?

Typical onset of Corpus callosum agenesis-macrocephaly-hypertelorism syndrome is neonatal. Age of onset can vary across affected individuals.