Susac syndrome

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Overview

Susac syndrome (also called retinocochleocerebral vasculopathy or RED-M syndrome) is a rare autoimmune disease where the body's immune system mistakenly attacks the tiny blood vessels in three specific parts of the body: the brain, the retinas (the light-sensitive tissue at the back of the eyes), and the inner ears. This attack causes these small vessels to become inflamed and blocked, cutting off blood supply to these areas and causing damage over time. The three main areas affected lead to three main groups of symptoms: problems with thinking and memory (from brain involvement), vision loss (from eye involvement), and hearing loss with ringing in the ears (from inner ear involvement). Not everyone has all three problems at the same time, which can make the disease hard to diagnose early. It most commonly affects young adults, especially women between the ages of 16 and 40. Treatment focuses on calming the immune system using medications like corticosteroids (such as prednisone), intravenous immunoglobulin (IVIG), and other immune-suppressing drugs. With early and aggressive treatment, many people can recover significantly, though some may have lasting effects. The disease can come in episodes (flares) or follow a more continuous course.

Also known as:

Key symptoms:

Memory loss and confusionDifficulty thinking clearly or concentrating (brain fog)Hearing loss, often in both earsRinging or buzzing in the ears (tinnitus)Vision loss or blind spotsHeadachesSlurred speechPersonality or mood changesDizziness or balance problemsPsychiatric symptoms such as paranoia or depressionDifficulty walkingFatigue

Clinical phenotype terms (23)— hover any for plain English
Personality changesHP:0000751
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Susac syndrome.

View clinical trials →

No actively recruiting trials found for Susac syndrome at this time.

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

Search ClinicalTrials.gov ↗Join the Susac syndrome community →

No specialists are currently listed for Susac 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 Susac syndrome.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Susac syndrome

1 articles
NewsJ NEUROIMMUNOLMar 24, 2026
Retrospective treatment comparisons in patients with Susac syndrome
Published in J Neuroimmunol. Wurth S et al.
See all news about Susac syndrome

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.How certain are you about the diagnosis of Susac syndrome, and what tests confirmed it?,Which parts of my body — brain, eyes, or ears — are currently affected, and how severe is the damage?,What treatment plan do you recommend, and how long will I need to take immune-suppressing medications?,What are the signs that the disease is getting worse or coming back, and when should I go to the emergency room?,Are there any clinical trials I might be eligible for?,What long-term effects should I expect, and what can I do to protect my hearing and vision?,Should I see a cognitive specialist or neuropsychologist to help with memory and thinking problems?

Common questions about Susac syndrome

What is Susac syndrome?

Susac syndrome (also called retinocochleocerebral vasculopathy or RED-M syndrome) is a rare autoimmune disease where the body's immune system mistakenly attacks the tiny blood vessels in three specific parts of the body: the brain, the retinas (the light-sensitive tissue at the back of the eyes), and the inner ears. This attack causes these small vessels to become inflamed and blocked, cutting off blood supply to these areas and causing damage over time. The three main areas affected lead to three main groups of symptoms: problems with thinking and memory (from brain involvement), vision loss

How is Susac syndrome inherited?

Susac syndrome follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Susac syndrome typically begin?

Typical onset of Susac syndrome is adult. Age of onset can vary across affected individuals.