Microphthalmia-brain atrophy syndrome

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ORPHA:77299OMIM:611222Q11.2
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8Treatment centers

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Overview

Microphthalmia-brain atrophy syndrome is a very rare genetic condition that affects both the eyes and the brain. 'Microphthalmia' means that one or both eyes are abnormally small, which can cause serious vision problems or even blindness. Along with small eyes, people with this syndrome also experience brain atrophy, which means parts of the brain shrink or do not develop properly over time. This leads to problems with thinking, learning, and movement. The condition is present from birth, though some features may become more noticeable as a child grows. Key symptoms include small or underdeveloped eyes, intellectual disability, delayed development of skills like walking and talking, and muscle tone problems. Seizures may also occur in some individuals. Brain imaging often shows visible changes in brain structure. There is currently no cure for microphthalmia-brain atrophy syndrome. Treatment focuses on managing symptoms and supporting development. This may include special education programs, physical therapy, occupational therapy, vision support, and medications to control seizures if they occur. A team of specialists is usually needed to provide the best care.

Also known as:

Key symptoms:

Abnormally small eyes (one or both)Reduced or absent visionBrain shrinkage (brain atrophy) visible on scansIntellectual disabilityDelayed speech and language developmentDelayed motor milestones such as sitting and walkingLow or abnormal muscle toneSeizuresSmall head size (microcephaly) in some casesFeeding difficulties in infancy

Clinical phenotype terms (20)— hover any for plain English
Bilateral microphthalmosHP:0007633Diffuse cerebral atrophyHP:0002506Lateral ventricle dilatationHP:0006956Diffuse demyelination of the cerebral white matterHP:0007162Atrophy/Degeneration affecting the brainstemHP:0007366Corpus callosum atrophyHP:0007371Inappropriate cryingHP:0030215Multifocal seizuresHP:0031165Abnormal pons morphologyHP:0007361Focal hyperkinetic seizureHP:0011174Vegetative stateHP:0031358Tongue thrustingHP:0100703
Inheritance

X-linked recessive

Carried on the X chromosome; typically affects males more than females

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 Microphthalmia-brain atrophy syndrome.

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No actively recruiting trials found for Microphthalmia-brain atrophy syndrome at this time.

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No specialists are currently listed for Microphthalmia-brain atrophy 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 Microphthalmia-brain atrophy syndrome.

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Community

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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 genetic testing do you recommend to confirm the diagnosis, and should other family members be tested?,What therapies should we start right away to support my child's development?,How often should my child see an eye specialist, and what vision supports are available?,What are the signs that my child's condition may be getting worse, and when should I seek emergency care?,Are there any clinical trials or research studies we could participate in?,What educational supports should we ask for at school?,What does the long-term outlook look like, and what kind of support will my child need as an adult?

Common questions about Microphthalmia-brain atrophy syndrome

What is Microphthalmia-brain atrophy syndrome?

Microphthalmia-brain atrophy syndrome is a very rare genetic condition that affects both the eyes and the brain. 'Microphthalmia' means that one or both eyes are abnormally small, which can cause serious vision problems or even blindness. Along with small eyes, people with this syndrome also experience brain atrophy, which means parts of the brain shrink or do not develop properly over time. This leads to problems with thinking, learning, and movement. The condition is present from birth, though some features may become more noticeable as a child grows. Key symptoms include small or underdeve

How is Microphthalmia-brain atrophy syndrome inherited?

Microphthalmia-brain atrophy syndrome follows a x-linked recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Microphthalmia-brain atrophy syndrome typically begin?

Typical onset of Microphthalmia-brain atrophy syndrome is neonatal. Age of onset can vary across affected individuals.