Overview
Progressive bifocal chorioretinal atrophy (PBCRA) is a rare inherited eye disease that slowly damages the retina and choroid — the light-sensitive layers at the back of the eye. The disease causes two distinct areas of tissue loss (called atrophy) in each eye: one near the center of vision (the macula) and one toward the outer edge of the retina (the nasal periphery). These patches of damage grow larger over time, which is why the disease is called 'progressive.' PBCRA is also sometimes referred to by its full descriptive name in older medical literature. People with PBCRA typically notice problems with their central vision first, making it harder to read, recognize faces, or see fine details. Side (peripheral) vision may also be affected as the disease advances. Night vision difficulties and sensitivity to light are also common. The condition affects both eyes and tends to worsen gradually over decades. There is currently no cure or approved treatment that can stop or reverse the damage caused by PBCRA. Care focuses on helping people make the most of their remaining vision through low vision aids, magnification devices, and lifestyle adjustments. Regular monitoring by an eye specialist is important to track how the disease is progressing and to plan for future vision needs.
Key symptoms:
Blurred or reduced central visionDifficulty reading or seeing fine detailsBlind spots in the center of visionLoss of side (peripheral) vision over timePoor vision in dim light or at nightSensitivity to bright lightDifficulty recognizing facesGradual worsening of vision over yearsPale or washed-out patches visible on eye examination
Clinical phenotype terms (7)— hover any for plain English
Autosomal dominant
Passed on from just one parent; each child has about a 50% chance of inheriting it
Childhood
Begins in childhood, roughly ages 1 to 12
Treatments
No FDA-approved treatments are currently listed for Progressive bifocal chorioretinal atrophy.
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Specialists
View all specialists →No specialists are currently listed for Progressive bifocal chorioretinal atrophy.
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Progressive bifocal chorioretinal atrophy.
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Caregiver Resources
NORD Caregiver Resources
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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 quickly do you expect my vision to change, and what signs should I watch for?,Should other members of my family be tested for this condition?,What low vision services or rehabilitation programs do you recommend?,Are there any clinical trials or research studies I could participate in?,What accommodations should I request at school or work?,Are there any vitamins, supplements, or lifestyle changes that might help protect my remaining vision?,How often should I have my eyes checked, and what tests will be done at each visit?
Common questions about Progressive bifocal chorioretinal atrophy
What is Progressive bifocal chorioretinal atrophy?
Progressive bifocal chorioretinal atrophy (PBCRA) is a rare inherited eye disease that slowly damages the retina and choroid — the light-sensitive layers at the back of the eye. The disease causes two distinct areas of tissue loss (called atrophy) in each eye: one near the center of vision (the macula) and one toward the outer edge of the retina (the nasal periphery). These patches of damage grow larger over time, which is why the disease is called 'progressive.' PBCRA is also sometimes referred to by its full descriptive name in older medical literature. People with PBCRA typically notice pr
How is Progressive bifocal chorioretinal atrophy inherited?
Progressive bifocal chorioretinal atrophy follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Progressive bifocal chorioretinal atrophy typically begin?
Typical onset of Progressive bifocal chorioretinal atrophy is childhood. Age of onset can vary across affected individuals.