OBSOLETE: Rare disease with corpus callosum agenesis associated with peripheral neuropathy

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ORPHA:207031
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Overview

This entry, previously known as 'Rare disease with corpus callosum agenesis associated with peripheral neuropathy,' has been marked as obsolete in the Orphanet classification system. This means the condition has been reclassified, merged with another diagnosis, or its classification has been updated based on newer scientific understanding. The original description referred to a rare condition in which a person is born without or with an underdeveloped corpus callosum — the thick band of nerve fibers that connects the left and right halves of the brain — combined with peripheral neuropathy, which is damage to the nerves outside the brain and spinal cord. Symptoms of corpus callosum agenesis can include developmental delays, intellectual disability, seizures, and problems with coordination. Peripheral neuropathy adds symptoms like numbness, tingling, weakness, or pain in the hands and feet. Because this entry is now obsolete, patients and families who were previously given this diagnosis should speak with their clinical geneticist or neurologist to determine the current, updated diagnosis that applies to their condition. The updated classification may fall under a more specific genetic syndrome, such as hereditary motor and sensory neuropathy with agenesis of the corpus callosum (also known as Andermann syndrome or ACCPN), or another related condition. Treatment has generally been supportive and symptom-based, focusing on managing seizures, physical therapy for motor difficulties, and addressing neuropathy symptoms.

Key symptoms:

Absence or underdevelopment of the connection between brain halvesNumbness or tingling in hands and feetMuscle weakness, especially in the legsDelayed motor milestones like sitting and walkingIntellectual disability or learning difficultiesSeizuresPoor coordination and balanceDifficulty with fine motor tasksProgressive difficulty walkingMuscle wastingAbnormal eye movementsScoliosis or spinal curvatureSpeech delays

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 ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Rare disease with corpus callosum agenesis associated with peripheral neuropathy.

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No actively recruiting trials found for OBSOLETE: Rare disease with corpus callosum agenesis associated with peripheral neuropathy at this time.

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No specialists are currently listed for OBSOLETE: Rare disease with corpus callosum agenesis associated with peripheral neuropathy.

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 OBSOLETE: Rare disease with corpus callosum agenesis associated with peripheral neuropathy.

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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.Has my diagnosis been updated from this obsolete classification, and what is the current name for my condition?,Which gene is responsible, and should my family members be tested?,What is the expected progression of symptoms over time?,What therapies are available to help maintain mobility and function?,Are there any clinical trials or new treatments being studied for this condition?,What seizure management plan should we follow?,What support services and resources are available for our family?

Common questions about OBSOLETE: Rare disease with corpus callosum agenesis associated with peripheral neuropathy

What is OBSOLETE: Rare disease with corpus callosum agenesis associated with peripheral neuropathy?

This entry, previously known as 'Rare disease with corpus callosum agenesis associated with peripheral neuropathy,' has been marked as obsolete in the Orphanet classification system. This means the condition has been reclassified, merged with another diagnosis, or its classification has been updated based on newer scientific understanding. The original description referred to a rare condition in which a person is born without or with an underdeveloped corpus callosum — the thick band of nerve fibers that connects the left and right halves of the brain — combined with peripheral neuropathy, whi

How is OBSOLETE: Rare disease with corpus callosum agenesis associated with peripheral neuropathy inherited?

OBSOLETE: Rare disease with corpus callosum agenesis associated with peripheral neuropathy follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does OBSOLETE: Rare disease with corpus callosum agenesis associated with peripheral neuropathy typically begin?

Typical onset of OBSOLETE: Rare disease with corpus callosum agenesis associated with peripheral neuropathy is infantile. Age of onset can vary across affected individuals.