COL4A1/2-related familial vascular leukoencephalopathy

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ORPHA:36383OMIM:175780I67.8
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Overview

COL4A1/COL4A2-related familial vascular leukoencephalopathy is a rare inherited condition that affects the small blood vessels in the brain and other organs. It is caused by changes (mutations) in the COL4A1 or COL4A2 genes, which provide instructions for making a protein called type IV collagen. This protein is a key building block of the thin membranes that surround and support blood vessels throughout the body. When this protein does not work properly, blood vessel walls become fragile and prone to damage. The brain is the most affected organ. White matter — the tissue that connects different parts of the brain — becomes damaged over time, which is why this condition is sometimes called a 'leukoencephalopathy' (leuko means white, encephalo means brain). People may also experience small strokes, bleeding in the brain, and damage to the eyes and kidneys. Symptoms can vary widely even within the same family. Common symptoms include strokes or stroke-like episodes (sometimes in young adults or even children), migraine headaches, muscle weakness or stiffness, problems with thinking and memory, and vision difficulties. Some people also develop kidney disease or muscle problems. There is currently no cure, and treatment focuses on managing symptoms, reducing stroke risk, and protecting affected organs. Blood pressure control is especially important. This condition is also sometimes referred to as COL4A1-related brain small vessel disease or COL4A2-related cerebrovascular disease.

Also known as:

Key symptoms:

Stroke or stroke-like episodes, sometimes at a young ageMigraine headaches, often with visual disturbances (aura)Weakness or stiffness on one side of the bodyProblems with memory, thinking, or concentrationVision problems, including double vision or vision lossSeizuresDifficulty walking or keeping balanceBleeding inside the brain (intracerebral hemorrhage)Kidney problems (blood or protein in the urine)Muscle cramps or weaknessCataracts or other eye abnormalitiesRaynaud's phenomenon (fingers turning white or blue in the cold)Abnormal heart rhythm in some cases

Inheritance

Autosomal dominant

Passed on from just one parent; each child has about a 50% chance of inheriting it

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗OMIM ↗NORD ↗

FDA & Trial Timeline

1 event
Oct 1999

Aromasin: FDA approved

Treatment of advanced breast cancer in postmenopausal women whose disease has progressed following tamoxifen therapy.

FDAcompleted

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for COL4A1/2-related familial vascular leukoencephalopathy.

View clinical trials →

No actively recruiting trials found for COL4A1/2-related familial vascular leukoencephalopathy at this time.

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

Search ClinicalTrials.gov ↗Join the COL4A1/2-related familial vascular leukoencephalopathy community →

No specialists are currently listed for COL4A1/2-related familial vascular leukoencephalopathy.

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

Financial Resources

1 resources

Aromasin

Pharmacia & Upjohn

Aromasin — Contact Pharmacia & Upjohn

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Patient Assistance
Manufacturer Program
Accepting applications

Travel Grants

No travel grants are currently matched to COL4A1/2-related familial vascular leukoencephalopathy.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about COL4A1/2-related familial vascular leukoencephalopathy

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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.Which gene mutation do I have, and what does that mean for my specific risk of stroke or other complications?,Should my children or siblings be tested, and at what age?,What blood pressure target should I aim for, and which medications are safest for me?,Are there any activities or medications I should avoid because of my condition?,How often should I have brain MRI scans, eye exams, and kidney function tests?,Is there a clinical trial or research study I could join?,What should I do — and what should my family do — if I show signs of a stroke?

Common questions about COL4A1/2-related familial vascular leukoencephalopathy

What is COL4A1/2-related familial vascular leukoencephalopathy?

COL4A1/COL4A2-related familial vascular leukoencephalopathy is a rare inherited condition that affects the small blood vessels in the brain and other organs. It is caused by changes (mutations) in the COL4A1 or COL4A2 genes, which provide instructions for making a protein called type IV collagen. This protein is a key building block of the thin membranes that surround and support blood vessels throughout the body. When this protein does not work properly, blood vessel walls become fragile and prone to damage. The brain is the most affected organ. White matter — the tissue that connects differ

How is COL4A1/2-related familial vascular leukoencephalopathy inherited?

COL4A1/2-related familial vascular leukoencephalopathy follows a autosomal dominant inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

What treatment and support options exist for COL4A1/2-related familial vascular leukoencephalopathy?

1 patient support program are currently tracked on UniteRare for COL4A1/2-related familial vascular leukoencephalopathy. See the treatments and support programs sections for copay assistance, eligibility, and contact details.