Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrom

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ORPHA:500159OMIM:617751Q87.0
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Overview

Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrome is a very rare brain development disorder that affects children from birth. The name describes the main features: microcephaly means the head and brain are smaller than usual; corpus callosum hypoplasia means the band of nerve fibers connecting the two sides of the brain did not fully develop; cerebellar vermis hypoplasia means the lower back part of the brain (which controls balance and coordination) is underdeveloped; facial dysmorphism refers to distinctive facial features; and intellectual disability means learning and thinking are affected. This condition is sometimes referred to by its Orphanet code ORPHA:500159. Because the brain does not develop normally before birth, children with this syndrome typically show signs early in life, including a smaller-than-normal head size, delayed milestones like sitting and walking, and challenges with learning and communication. Seizures and problems with muscle tone are also commonly reported. Facial features may include a distinctive appearance that a specialist can recognize. There is currently no cure for this syndrome. Care focuses on managing symptoms, supporting development, and improving quality of life. This includes therapies such as physical, occupational, and speech therapy, as well as medications to control seizures if they occur. A team of specialists works together to support the child and family.

Key symptoms:

Smaller than normal head size (microcephaly)Intellectual disability and learning difficultiesDelayed developmental milestones such as sitting, walking, and talkingUnderdeveloped connection between the two sides of the brain (corpus callosum hypoplasia)Underdeveloped lower back part of the brain affecting balance and coordination (cerebellar vermis hypoplasia)Distinctive facial featuresSeizuresLow or high muscle toneDifficulty with speech and communicationPoor coordination and balance problems

Clinical phenotype terms (50)— hover any for plain English
Abnormal earlobe morphologyHP:0000363
Inheritance

Autosomal recessive

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

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrom.

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No actively recruiting trials found for Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrom at this time.

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No specialists are currently listed for Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrom.

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 Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrom.

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Community

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Latest news about Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrom

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Caregiver Resources

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.What genetic tests should my child have, and what might the results tell us about the cause?,What therapies should we start right away, and how often should my child attend them?,What signs of seizures should I watch for, and when should I call for emergency help?,What educational support and early intervention programs are available for my child?,Are there any clinical trials or research studies we could consider joining?,What is the likely progression of this condition as my child grows older?,Are other family members at risk, and should they be tested?

Common questions about Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrom

What is Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrom?

Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrome is a very rare brain development disorder that affects children from birth. The name describes the main features: microcephaly means the head and brain are smaller than usual; corpus callosum hypoplasia means the band of nerve fibers connecting the two sides of the brain did not fully develop; cerebellar vermis hypoplasia means the lower back part of the brain (which controls balance and coordination) is underdeveloped; facial dysmorphism refers to distinctive facial features; and

How is Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrom inherited?

Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrom follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrom typically begin?

Typical onset of Microcephaly-corpus callosum and cerebellar vermis hypoplasia-facial dysmorphism-intellectual disability syndrom is neonatal. Age of onset can vary across affected individuals.