Microcephalic cortical malformations-short stature due to RTTN deficiency

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ORPHA:468631OMIM:614833Q02
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Overview

Microcephalic cortical malformations with short stature due to RTTN deficiency is an extremely rare genetic condition that affects brain development and overall growth. The disease is caused by mutations in the RTTN gene, which provides instructions for making a protein called rotatin. This protein plays an important role in cell division and brain development before birth. Children born with this condition have an abnormally small head (microcephaly) because the brain does not develop properly. The brain's outer layer, called the cortex, shows structural abnormalities (cortical malformations) that can include simplified folding patterns or other irregularities. These brain changes typically lead to intellectual disability, developmental delays, and often seizures. Affected children also have notably short stature compared to their peers. Other features may include movement difficulties, speech delays, and sometimes vision problems. The severity can vary between individuals, but most children require significant support throughout their lives. There is currently no cure for this condition. Treatment focuses on managing symptoms, such as controlling seizures with medication, providing physical and occupational therapy, and supporting developmental progress. A team of specialists is usually needed to address the various aspects of this complex condition.

Key symptoms:

Abnormally small head size (microcephaly)Short stature or poor growthIntellectual disabilityDelayed development of motor skills like sitting and walkingSeizures or epilepsySpeech and language delaysAbnormal brain structure visible on MRIDifficulty with coordination and balanceMuscle stiffness or spasticityFeeding difficulties in infancyVision problemsSimplified brain folding patternsLow muscle tone in early life

Clinical phenotype terms (50)— hover any for plain English
Severe failure to thriveHP:0001525Pelvic kidneyHP:0000125Abnormality of the orbital regionHP:0000315
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 Microcephalic cortical malformations-short stature due to RTTN deficiency.

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No specialists are currently listed for Microcephalic cortical malformations-short stature due to RTTN deficiency.

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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

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Caregiver Resources

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific brain abnormalities does my child have, and how might they affect development?,What seizure medications are recommended, and what side effects should I watch for?,What therapies (physical, occupational, speech) should we start, and how often?,Are there any clinical trials or research studies for RTTN-related conditions that we could participate in?,What is the expected developmental trajectory for my child based on the severity of their condition?,Should we see a pediatric endocrinologist about my child's growth, and is growth hormone therapy an option?,What genetic counseling is available for our family regarding future pregnancies?

Common questions about Microcephalic cortical malformations-short stature due to RTTN deficiency

What is Microcephalic cortical malformations-short stature due to RTTN deficiency?

Microcephalic cortical malformations with short stature due to RTTN deficiency is an extremely rare genetic condition that affects brain development and overall growth. The disease is caused by mutations in the RTTN gene, which provides instructions for making a protein called rotatin. This protein plays an important role in cell division and brain development before birth. Children born with this condition have an abnormally small head (microcephaly) because the brain does not develop properly. The brain's outer layer, called the cortex, shows structural abnormalities (cortical malformations

How is Microcephalic cortical malformations-short stature due to RTTN deficiency inherited?

Microcephalic cortical malformations-short stature due to RTTN deficiency follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Microcephalic cortical malformations-short stature due to RTTN deficiency typically begin?

Typical onset of Microcephalic cortical malformations-short stature due to RTTN deficiency is neonatal. Age of onset can vary across affected individuals.