Microlissencephaly-micromelia syndrome

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Overview

Microlissencephaly-micromelia syndrome is an extremely rare and severe brain development disorder that is present from birth. The name describes its two main features: 'microlissencephaly' means the brain is both unusually small (microcephaly) and has a smooth surface instead of the normal folds and grooves (lissencephaly), and 'micromelia' means the arms and legs are abnormally short. Together, these features point to a problem that affects how the brain and limbs grow during pregnancy. The condition is also sometimes referred to simply as microlissencephaly with micromelia. Babies born with this syndrome typically have a very small head, a smooth brain with little or no folding, and shortened limbs. The brain abnormalities lead to profound intellectual disability, very limited or absent voluntary movement, and often severe seizures. Most affected infants also have feeding difficulties and may need breathing support. The condition is thought to be genetic in origin, though the exact gene or genes responsible have not been fully confirmed in all reported cases. There is currently no cure for microlissencephaly-micromelia syndrome. Care focuses on managing symptoms, keeping the child comfortable, preventing complications like aspiration pneumonia, and supporting the family. The prognosis is very serious, and most affected children do not survive beyond early infancy or childhood. Families benefit greatly from palliative care teams, genetic counseling, and strong support networks.

Also known as:

Key symptoms:

Abnormally small head size (microcephaly)Smooth brain surface with absent or reduced folding (lissencephaly)Unusually short arms and legs (micromelia)Profound intellectual disabilityLittle or no voluntary movementSevere seizuresFeeding difficulties and poor sucking reflexBreathing problems requiring supportVery low muscle tone (hypotonia)Absent or very limited response to surroundingsAbnormal facial featuresFailure to reach developmental milestones

Clinical phenotype terms (25)— hover any for plain English
HypoparathyroidismHP:000082911 pairs of ribsHP:0000878LissencephalyHP:0001339Generalized hypertrichosisHP:0004554
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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Microlissencephaly-micromelia syndrome.

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No actively recruiting trials found for Microlissencephaly-micromelia syndrome at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Microlissencephaly-micromelia syndrome community →

No specialists are currently listed for Microlissencephaly-micromelia 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 Microlissencephaly-micromelia syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Microlissencephaly-micromelia syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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 genetic tests should we do to find the cause, and what could the results mean for future pregnancies?,What seizure medications are most appropriate, and what should we do if a seizure does not stop?,Does my child need a feeding tube, and what are the risks and benefits?,What signs should prompt us to go to the emergency room immediately?,What does palliative care involve, and when should we start?,Are there any research studies or registries we can participate in?,What support services are available for our family, including siblings and mental health support?

Common questions about Microlissencephaly-micromelia syndrome

What is Microlissencephaly-micromelia syndrome?

Microlissencephaly-micromelia syndrome is an extremely rare and severe brain development disorder that is present from birth. The name describes its two main features: 'microlissencephaly' means the brain is both unusually small (microcephaly) and has a smooth surface instead of the normal folds and grooves (lissencephaly), and 'micromelia' means the arms and legs are abnormally short. Together, these features point to a problem that affects how the brain and limbs grow during pregnancy. The condition is also sometimes referred to simply as microlissencephaly with micromelia. Babies born with

How is Microlissencephaly-micromelia syndrome inherited?

Microlissencephaly-micromelia syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Microlissencephaly-micromelia syndrome typically begin?

Typical onset of Microlissencephaly-micromelia syndrome is neonatal. Age of onset can vary across affected individuals.