Occipital pachygyria and polymicrogyria

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ORPHA:280640OMIM:614115Q04.3
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Overview

Occipital pachygyria and polymicrogyria is a rare brain malformation that affects the back part of the brain, called the occipital lobe. In this condition, the normal folds of the brain (called gyri) do not form correctly during development before birth. "Pachygyria" means the brain folds are too thick and too few, while "polymicrogyria" means there are too many small, abnormally formed folds. When these changes occur in the occipital region, they primarily affect the area of the brain responsible for processing vision. People with this condition may experience a range of symptoms depending on how much of the brain is affected. Common problems include visual difficulties, seizures (epilepsy), developmental delays, and varying degrees of intellectual disability. Some individuals may have relatively mild symptoms, while others can be more severely affected. Seizures often begin in infancy or early childhood and may be difficult to control with medication. There is currently no cure for this condition. Treatment focuses on managing symptoms, particularly controlling seizures with anti-epileptic medications, and providing supportive therapies such as physical therapy, occupational therapy, and speech therapy. Early intervention programs can help children reach their developmental potential. The outlook varies widely depending on the extent of the brain malformation and how well seizures respond to treatment.

Also known as:

Key symptoms:

Seizures or epilepsyVision problems or visual impairmentDevelopmental delaysIntellectual disabilityDifficulty with motor skillsDelayed speech and language developmentLow muscle tone in infancyDifficulty with fine motor tasks like writing or graspingLearning difficultiesProblems with balance and coordinationCortical visual impairmentFeeding difficulties in infancy

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

1 available

Dyural 80-Lm

METHYLPREDNISOLONE ACETATE, LIDOCAINE HYDROCHLORIDE, BUPIVACAINE HYDROCHLORIDE, POVIDINE IODINE, ISOPROPYL ALCOHOL· Advanced Rx Pharmacy of Tennessee, LLC■ Boxed Warning
Control of severe or incapacitating allergic conditions intractable to adequate trials of conventional treatment in asthma, atopic dermatitis, contact dermatitis, drug hypersensitivity reactions, seru

Control of severe or incapacitating allergic conditions intractable to adequate trials of conventional treatment in asthma, atopic dermatitis, contact dermatitis, drug hypersensitivity reactions, serum sickness, transfusion reactions

No actively recruiting trials found for Occipital pachygyria and polymicrogyria at this time.

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

Search ClinicalTrials.gov ↗Join the Occipital pachygyria and polymicrogyria community →

No specialists are currently listed for Occipital pachygyria and polymicrogyria.

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 Occipital pachygyria and polymicrogyria.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Occipital pachygyria and polymicrogyria

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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.How much of my child's brain is affected, and what does the MRI show specifically?,What type of seizures does my child have, and what is the best medication to control them?,Should we pursue genetic testing, and what might the results tell us?,What therapies do you recommend, and how often should they occur?,What is the long-term outlook for my child's development and independence?,Are there any clinical trials or new treatments being studied for this condition?,What should I do if my child has a seizure that lasts more than 5 minutes?

Common questions about Occipital pachygyria and polymicrogyria

What is Occipital pachygyria and polymicrogyria?

Occipital pachygyria and polymicrogyria is a rare brain malformation that affects the back part of the brain, called the occipital lobe. In this condition, the normal folds of the brain (called gyri) do not form correctly during development before birth. "Pachygyria" means the brain folds are too thick and too few, while "polymicrogyria" means there are too many small, abnormally formed folds. When these changes occur in the occipital region, they primarily affect the area of the brain responsible for processing vision. People with this condition may experience a range of symptoms depending o

At what age does Occipital pachygyria and polymicrogyria typically begin?

Typical onset of Occipital pachygyria and polymicrogyria is infantile. Age of onset can vary across affected individuals.