Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome

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Overview

Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome is an extremely rare genetic condition that affects the brain and eyes. The disease is caused by mutations in a gene that leads to damage of the white matter in the brain (called leukoencephalopathy), strokes caused by blocked blood vessels (ischemic strokes), and a progressive eye condition called retinitis pigmentosa that causes gradual vision loss. The white matter damage in the brain can lead to problems with thinking, movement, and coordination. Ischemic strokes can occur at unusually young ages and may cause sudden weakness, speech difficulties, or other neurological problems. Retinitis pigmentosa typically starts with difficulty seeing in dim light (night blindness) and gradually narrows the field of vision over time. Because this condition affects multiple organ systems — primarily the brain and eyes — patients often need care from several different specialists. There is currently no cure for this syndrome, and treatment focuses on managing symptoms, preventing strokes, and supporting vision. The condition follows an autosomal recessive inheritance pattern, meaning a child must inherit a faulty copy of the gene from both parents to develop the disease.

Key symptoms:

Night blindnessProgressive vision lossNarrowing of the visual field (tunnel vision)Stroke at a young ageSudden weakness on one side of the bodyDifficulty speaking or understanding speechWhite matter changes in the brainProblems with thinking and memoryDifficulty with coordination and balanceHeadachesMuscle stiffness or spasticityFatigueDifficulty walking

Inheritance

Autosomal recessive

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

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome.

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No actively recruiting trials found for Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome at this time.

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No specialists are currently listed for Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa 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 Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome.

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Community

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Latest news about Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome

1 articles
Clinical trialUNITERAREApr 3, 2026
New Recruiting Trial: Application of Digital Twins' Technology in Patients Who Had a Stroke, With Moyamoya Disease and With Cerebral Amyloid Angiopathy (CAA) During the Secondary Prevention Phase: A Proof of Concept Using a Randomized Control Trial (Clinical Study 6, STRATIF-AI Project)
Researchers are testing a new technology called 'digital twins' to help prevent second strokes in patients with three rare brain conditions: moyamoya disease, c
See all news about Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome

Caregiver Resources

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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 specific genetic mutation is causing this condition in my case?,How can we best prevent future strokes?,What is the expected rate of vision loss, and what can we do to support my remaining vision?,Are there any clinical trials or research studies I could participate in?,What therapies (physical, occupational, speech) would benefit me most right now?,How often should I have brain MRI scans and eye exams?,Should my family members be tested to see if they are carriers?

Common questions about Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome

What is Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome?

Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome is an extremely rare genetic condition that affects the brain and eyes. The disease is caused by mutations in a gene that leads to damage of the white matter in the brain (called leukoencephalopathy), strokes caused by blocked blood vessels (ischemic strokes), and a progressive eye condition called retinitis pigmentosa that causes gradual vision loss. The white matter damage in the brain can lead to problems with thinking, movement, and coordination. Ischemic strokes can occur at unusually young ages and may

How is Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome inherited?

Autosomal recessive leukoencephalopathy-ischemic stroke-retinitis pigmentosa syndrome follows a autosomal recessive inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.