Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay syndrome

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ORPHA:505237OMIM:617452Q87.8
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8Treatment centers

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Overview

Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay syndrome is a very rare genetic condition that affects the brain, limbs, and facial features from birth or early infancy. It is sometimes referred to by its Orphanet code ORPHA:505237. The condition is caused by changes (mutations) in a specific gene that disrupt normal development before and after birth. Children with this syndrome typically experience seizures that begin very early in life, often in the first weeks or months. They also have differences in the shape or structure of their hands and feet (called distal limb anomalies), along with distinctive facial features that doctors can recognize. Global developmental delay means that children reach milestones like sitting, walking, and talking much later than expected, or may not reach some milestones at all. There is currently no cure for this condition. Treatment focuses on managing symptoms, especially controlling seizures with anti-seizure medications, and supporting development through physical, occupational, and speech therapy. A team of specialists works together to help affected children and their families manage day-to-day challenges and improve quality of life.

Key symptoms:

Seizures starting in the first weeks or months of lifeDelayed development (sitting, walking, talking later than expected)Intellectual disabilityUnusual hand or foot shape (such as short fingers or toe abnormalities)Distinctive facial features (such as widely spaced eyes, a flat nasal bridge, or low-set ears)Low muscle tone (floppiness) in infancyFeeding difficulties in early lifeLimited or absent speechBehavioral challenges

Clinical phenotype terms (50)— hover any for plain English
Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

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 Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay syndrome.

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No actively recruiting trials found for Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay syndrome at this time.

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No specialists are currently listed for Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay 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 Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay 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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Family & Caregiver Grants

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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.Which specific gene is thought to be causing my child's condition, and what genetic tests should we do?,What type of seizures does my child have, and which anti-seizure medication is the best starting point?,What therapies (physical, occupational, speech) should my child start, and how often?,Are there any clinical trials or research studies we could participate in?,What should we do if my child has a seizure that lasts more than 5 minutes?,What developmental milestones should we realistically hope for, and how can we best support progress?,Should other family members be tested, and what is the chance of this happening in a future pregnancy?

Common questions about Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay syndrome

What is Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay syndrome?

Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay syndrome is a very rare genetic condition that affects the brain, limbs, and facial features from birth or early infancy. It is sometimes referred to by its Orphanet code ORPHA:505237. The condition is caused by changes (mutations) in a specific gene that disrupt normal development before and after birth. Children with this syndrome typically experience seizures that begin very early in life, often in the first weeks or months. They also have differences in the shape or structure of their hands and feet (

How is Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay syndrome inherited?

Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay syndrome follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay syndrome typically begin?

Typical onset of Early-onset seizures-distal limb anomalies-facial dysmorphism-global developmental delay syndrome is neonatal. Age of onset can vary across affected individuals.