C11ORF73-related autosomal recessive hypomyelinating leukodystrophy

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ORPHA:495844OMIM:616881G93.8
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Overview

C11ORF73-related autosomal recessive hypomyelinating leukodystrophy is a rare inherited brain disease. It is sometimes referred to by the gene name involved: C11ORF73-related leukodystrophy. The disease belongs to a group of conditions called leukodystrophies, which affect the white matter of the brain. White matter is made up of nerve fibers coated in a protective layer called myelin. Myelin acts like insulation on an electrical wire — it helps signals travel quickly and correctly through the brain and nervous system. In this disease, myelin does not form properly from early in life, a problem called hypomyelination. Because the brain's communication system is disrupted, children with this condition typically experience delays in reaching developmental milestones, problems with movement and coordination, muscle tone abnormalities, and intellectual disability. Symptoms usually appear in infancy or early childhood. Brain MRI scans show a characteristic pattern of reduced or absent myelin. There is currently no cure for this disease. Treatment focuses on managing symptoms, supporting development, and improving quality of life. This includes physical therapy, occupational therapy, speech therapy, and medications to manage specific symptoms such as seizures. A team of specialists is usually needed to provide the best care.

Also known as:

Key symptoms:

Delayed motor development (late sitting, standing, or walking)Intellectual disability or learning difficultiesPoor muscle tone (hypotonia) or abnormally stiff muscles (spasticity)Problems with coordination and balanceNystagmus (involuntary, repetitive eye movements)SeizuresSpeech and language delaysDifficulty swallowingSlow or absent development of normal reflexesProgressive neurological decline in some cases

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 C11ORF73-related autosomal recessive hypomyelinating leukodystrophy.

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No actively recruiting trials found for C11ORF73-related autosomal recessive hypomyelinating leukodystrophy at this time.

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No specialists are currently listed for C11ORF73-related autosomal recessive hypomyelinating leukodystrophy.

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 C11ORF73-related autosomal recessive hypomyelinating leukodystrophy.

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Community

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Latest news about C11ORF73-related autosomal recessive hypomyelinating leukodystrophy

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Caregiver Resources

NORD Caregiver Resources

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Mental Health Support

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Family & Caregiver Grants

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Social Security Disability

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Questions for your doctor

Bring these to your next appointment

  • Q1.What specific changes were found in the C11ORF73 gene, and what do they mean for my child's prognosis?,Should other family members be tested as carriers?,What therapies do you recommend starting right away, and how often should they happen?,Are there any clinical trials or research studies we should know about?,What signs should prompt us to go to the emergency room?,How often should my child have brain MRI scans to monitor the disease?,Are there leukodystrophy specialist centers or expert networks you would recommend we connect with?

Common questions about C11ORF73-related autosomal recessive hypomyelinating leukodystrophy

What is C11ORF73-related autosomal recessive hypomyelinating leukodystrophy?

C11ORF73-related autosomal recessive hypomyelinating leukodystrophy is a rare inherited brain disease. It is sometimes referred to by the gene name involved: C11ORF73-related leukodystrophy. The disease belongs to a group of conditions called leukodystrophies, which affect the white matter of the brain. White matter is made up of nerve fibers coated in a protective layer called myelin. Myelin acts like insulation on an electrical wire — it helps signals travel quickly and correctly through the brain and nervous system. In this disease, myelin does not form properly from early in life, a proble

How is C11ORF73-related autosomal recessive hypomyelinating leukodystrophy inherited?

C11ORF73-related autosomal recessive hypomyelinating leukodystrophy follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does C11ORF73-related autosomal recessive hypomyelinating leukodystrophy typically begin?

Typical onset of C11ORF73-related autosomal recessive hypomyelinating leukodystrophy is infantile. Age of onset can vary across affected individuals.