Intellectual disability-epilepsy-extrapyramidal syndrome

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ORPHA:468620OMIM:617171G93.8
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Overview

Intellectual disability-epilepsy-extrapyramidal syndrome is an extremely rare genetic neurological condition that affects brain development and function. The disease is characterized by three main features: intellectual disability (difficulty with learning, reasoning, and problem-solving), epilepsy (recurrent seizures), and extrapyramidal symptoms (movement problems such as involuntary movements, muscle stiffness, tremors, or difficulty coordinating movements). These extrapyramidal symptoms arise from problems in the parts of the brain that help control and coordinate movement. Symptoms typically begin in early life, and affected children may show delayed developmental milestones such as sitting, walking, and speaking later than expected. Seizures can vary in type and severity, and the movement difficulties may worsen over time. Some individuals may also experience behavioral challenges. There is currently no cure for this condition. Treatment focuses on managing symptoms. Anti-seizure medications are used to control epilepsy, while medications and therapies may help with movement problems. Physical therapy, occupational therapy, and speech therapy play important roles in supporting development and daily functioning. Early intervention with a multidisciplinary team of specialists is important to optimize quality of life. Because this is such a rare condition, management is often individualized based on each person's specific symptoms and needs.

Key symptoms:

Intellectual disability (difficulty learning and understanding)Seizures (epilepsy)Involuntary movementsMuscle stiffness or rigidityTremorsDifficulty coordinating movementsDelayed speech developmentDelayed motor milestones (sitting, walking)Abnormal postures (dystonia)Behavioral difficultiesPoor balanceDifficulty with fine motor skillsSlow or abnormal gait

Clinical phenotype terms (30)— hover any for plain English
Abnormality of pain sensationHP:0010832
Inheritance

Autosomal recessive

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

Age of Onset

Infantile

Begins in infancy, roughly 1 month to 2 years old

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Intellectual disability-epilepsy-extrapyramidal syndrome.

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No actively recruiting trials found for Intellectual disability-epilepsy-extrapyramidal syndrome at this time.

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No specialists are currently listed for Intellectual disability-epilepsy-extrapyramidal 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 Intellectual disability-epilepsy-extrapyramidal 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

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 specific genetic mutation is causing my child's condition, and what does it mean for their future?,What seizure medications are best suited for my child, and what side effects should I watch for?,Are there any treatments available for the movement problems?,What therapies (physical, occupational, speech) should we start, and how often?,Should other family members be tested for this genetic change?,Are there any clinical trials or research studies we could participate in?,What emergency plan should we have in place for prolonged seizures?

Common questions about Intellectual disability-epilepsy-extrapyramidal syndrome

What is Intellectual disability-epilepsy-extrapyramidal syndrome?

Intellectual disability-epilepsy-extrapyramidal syndrome is an extremely rare genetic neurological condition that affects brain development and function. The disease is characterized by three main features: intellectual disability (difficulty with learning, reasoning, and problem-solving), epilepsy (recurrent seizures), and extrapyramidal symptoms (movement problems such as involuntary movements, muscle stiffness, tremors, or difficulty coordinating movements). These extrapyramidal symptoms arise from problems in the parts of the brain that help control and coordinate movement. Symptoms typic

How is Intellectual disability-epilepsy-extrapyramidal syndrome inherited?

Intellectual disability-epilepsy-extrapyramidal syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Intellectual disability-epilepsy-extrapyramidal syndrome typically begin?

Typical onset of Intellectual disability-epilepsy-extrapyramidal syndrome is infantile. Age of onset can vary across affected individuals.