Pachygyria-intellectual disability-epilepsy syndrome

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ORPHA:2798OMIM:600176G40.4
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8Treatment centers

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Overview

Pachygyria-intellectual disability-epilepsy syndrome is a rare brain development disorder present from birth. The name 'pachygyria' comes from Greek words meaning 'thick' and 'gyrus' (the folds of the brain). In this condition, the brain does not form its normal folds and grooves during pregnancy. Instead, the brain surface appears smoother and thicker than usual. This abnormal brain structure leads to the three main features of the syndrome: intellectual disability (difficulty with learning and thinking), epilepsy (recurring seizures), and other neurological problems. The brain's unusual shape affects how nerve cells communicate with each other, which is why seizures and learning difficulties are so common. Seizures can range from mild to very severe and may be hard to control with medication. Children with this condition often have delayed development, meaning they may be slower to reach milestones like sitting, walking, and talking. Some children may never walk independently or develop spoken language. There is currently no cure for pachygyria-intellectual disability-epilepsy syndrome. Treatment focuses on managing symptoms, especially seizures, using anti-seizure medications. Supportive therapies such as physical therapy, speech therapy, and occupational therapy can help improve quality of life. Early intervention and a strong support team are very important for children and families living with this condition.

Also known as:

Key symptoms:

Recurring seizures (epilepsy)Intellectual disability (difficulty learning and thinking)Delayed development of motor skills like sitting and walkingDelayed or absent speech and language developmentMuscle tone problems (either too stiff or too floppy)Difficulty swallowing or feedingSmall head size (microcephaly) in some casesPoor coordination and balanceBehavioral challengesVision problems in some cases

Clinical phenotype terms (4)— hover any for plain English
Inheritance

Variable

Can be inherited in different ways depending on the underlying gene

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 Pachygyria-intellectual disability-epilepsy syndrome.

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No actively recruiting trials found for Pachygyria-intellectual disability-epilepsy syndrome at this time.

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No specialists are currently listed for Pachygyria-intellectual disability-epilepsy 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 Pachygyria-intellectual disability-epilepsy syndrome.

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Community

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Latest news about Pachygyria-intellectual disability-epilepsy syndrome

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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.Which gene is causing my child's condition, and what does that mean for their future?,What is the best anti-seizure medication for my child's type of epilepsy, and what are the side effects?,Should we consider the ketogenic diet for seizure control?,What therapies (physical, speech, occupational) should my child start, and how often?,What developmental milestones can we realistically hope for, and how can we support my child's progress?,Are there any clinical trials or research studies we should know about?,What should I do if my child has a prolonged seizure at home?

Common questions about Pachygyria-intellectual disability-epilepsy syndrome

What is Pachygyria-intellectual disability-epilepsy syndrome?

Pachygyria-intellectual disability-epilepsy syndrome is a rare brain development disorder present from birth. The name 'pachygyria' comes from Greek words meaning 'thick' and 'gyrus' (the folds of the brain). In this condition, the brain does not form its normal folds and grooves during pregnancy. Instead, the brain surface appears smoother and thicker than usual. This abnormal brain structure leads to the three main features of the syndrome: intellectual disability (difficulty with learning and thinking), epilepsy (recurring seizures), and other neurological problems. The brain's unusual sha

At what age does Pachygyria-intellectual disability-epilepsy syndrome typically begin?

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