Severe oculo-renal-cerebellar syndrome

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ORPHA:2715OMIM:257970Q04.8
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8Treatment centers

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Overview

Severe oculo-renal-cerebellar syndrome is a very rare condition that affects three main parts of the body: the eyes (oculo), the kidneys (renal), and the cerebellum — the part of the brain that controls balance and coordination (cerebellar). This condition is sometimes referred to as oculo-renal-cerebellar syndrome or ORCS. Because it affects multiple organ systems at once, it can cause a wide range of serious health problems from early in life. Children born with this syndrome typically have problems with their vision, including abnormal eye development or function. Their kidneys may not work properly, which can lead to problems filtering waste from the blood. The brain abnormalities, particularly in the cerebellum, can cause difficulties with movement, balance, and coordination. Intellectual disability and developmental delays are also commonly reported. Unfortunately, there is currently no cure for this condition. Treatment focuses on managing symptoms and supporting the affected organ systems. This may include kidney care, vision support, physical and occupational therapy, and educational support. A team of specialists is usually needed to provide the best care. Early diagnosis and a coordinated care plan can help improve quality of life for affected individuals and their families.

Also known as:

Key symptoms:

Vision problems or abnormal eye developmentKidney dysfunction or kidney failurePoor balance and coordinationIntellectual disabilityDevelopmental delaysAbnormal brain structure, especially in the cerebellumLow muscle tone (floppiness)Difficulty walking or movingAbnormal eye movementsProtein or blood in the urine

Clinical phenotype terms (29)— hover any for plain English
Abnormal retinal vascular morphologyHP:0008046GlomerulopathyHP:0100820Spastic diplegiaHP:0001264
Inheritance

Autosomal recessive

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

Age of Onset

Neonatal

Begins at or shortly after birth (first 4 weeks)

Orphanet ↗OMIM ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Severe oculo-renal-cerebellar syndrome.

View clinical trials →

No actively recruiting trials found for Severe oculo-renal-cerebellar syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Severe oculo-renal-cerebellar syndrome community →

No specialists are currently listed for Severe oculo-renal-cerebellar 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 Severe oculo-renal-cerebellar syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Severe oculo-renal-cerebellar syndrome

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

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 genetic tests should we do to confirm the diagnosis and understand the cause?,How severe is the kidney involvement, and what is the plan to protect kidney function long-term?,What therapies — physical, occupational, or speech — would benefit my child most right now?,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 we see each specialist, and who should coordinate my child's overall care?,Are other family members at risk, and should they be tested?

Common questions about Severe oculo-renal-cerebellar syndrome

What is Severe oculo-renal-cerebellar syndrome?

Severe oculo-renal-cerebellar syndrome is a very rare condition that affects three main parts of the body: the eyes (oculo), the kidneys (renal), and the cerebellum — the part of the brain that controls balance and coordination (cerebellar). This condition is sometimes referred to as oculo-renal-cerebellar syndrome or ORCS. Because it affects multiple organ systems at once, it can cause a wide range of serious health problems from early in life. Children born with this syndrome typically have problems with their vision, including abnormal eye development or function. Their kidneys may not wor

How is Severe oculo-renal-cerebellar syndrome inherited?

Severe oculo-renal-cerebellar syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Severe oculo-renal-cerebellar syndrome typically begin?

Typical onset of Severe oculo-renal-cerebellar syndrome is neonatal. Age of onset can vary across affected individuals.