National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Susac's Syndrome is not the name you expected.
Susac's syndrome is a rare disorder characterized by three main problems: impaired brain function (encephalopathy), partial or complete blockage (occlusion) of the arteries that supply blood to the retina (branch retinal artery occlusion, or BRAO), and inner ear disease (hearing loss, most notably).
Susac's syndrome is a rare disorder characterized by three main problems: impaired brain function (encephalopathy), partial or complete blockage (occlusion) of the small arteries that supply blood to the retina (branch retinal artery occlusion, or BRAO), and inner ear disease (hearing loss, most notably).
Three main forms of Susac's syndrome have become apparent. In one form, encephalopathy is the main problem. In the second form, BRAO and hearing loss are the main problems, and there is little or no brain disease. In the third form, encephalopathy is the main problem in the beginning, but recurrent episodes of BRAO and hearing loss become the main problem after the encephalopathy goes away.
The encephalopathic form of Susac's syndrome often resolves spontaneously (often going away within 1-3 years). The other forms tend to follow a more prolonged, more chronic course (3-10 years, or more). All forms require immunosuppressive treatment while the disease is active.
Although considered rare, Susac's syndrome is being recognized more often worldwide and its true frequency in the general population is unknown. This is because the disorder may be misdiagnosed, often as "atypical" multiple sclerosis.
Susac's syndrome is an autoimmune disease---specifically, an autoimmune endotheliopathy. "Autoimmune" means that a person's own immune system is mistakenly attacking one's own healthy tissue. An "endotheliopathy" is any disorder that involves injury to the endothelium, which is the thin layer of cells that line the inner walls of blood vessels. In Susac's syndrome, the person's own immune system is mistakenly attacking the endothelial lining of the smallest blood vessels (the capillaries, venules and arterioles) in the brain, retina, and inner ear. When the endothelial cells become injured, they tend to swell, and this endothelial cell swelling plays a key role in the partial or complete occlusion (blockage) of the tiny vessels in the brain, retina and inner ear. This blockage results in decreased blood flow through the vessels and, therefore, decreased delivery of oxygen and nutrients to the brain, retina, and inner ear---causing these three organs to suffer.
American Academy of Audiology
- 11480 Commerce Park Drive
- Suite 220
- Reston, VA 20191
- Tel: 703-790-8466
- Fax: 703-790-8631
- Tel: 800-222-2336
- Email: firstname.lastname@example.org
- Website: http://www.audiology.org
American Autoimmune & Related Diseases
- 22100 Gratiot Ave.
- Eastpointe, MI 48021
- Tel: (586)776-3900
- Fax: (586)776-3903
- Tel: (800)598-4668
- Email: email@example.com
- Website: http://www.aarda.org/
- Website: https://www.facebook.com/autoimmunityforum
Better Hearing Institute
- 1444 I Street NW
- Suite 700
- Washington, DC 20005
- United States
- Tel: (202)449-1100
- Fax: (703)684-6048
- Tel: (800)327-9355
- Email: firstname.lastname@example.org
- Website: http://www.betterhearing.org
Genetic and Rare Diseases (GARD) Information Center
- PO Box 8126
- Gaithersburg, MD 20898-8126
- Tel: (301)251-4925
- Fax: (301)251-4911
- Tel: (888)205-2311
- Website: http://rarediseases.info.nih.gov/GARD/
Hearing Loss Association of America
- 7910 Woodmont Avenue
- Suite 1200
- Bethesda, MD 20814
- Tel: (301)657-2248
- Fax: (301)913-9413
- Email: email@example.com
- Website: http://www.hearingloss.org
NIH/National Institute of Neurological Disorders and Stroke
- P.O. Box 5801
- Bethesda, MD 20824
- Tel: (301)496-5751
- Fax: (301)402-2186
- Tel: (800)352-9424
- Website: http://www.ninds.nih.gov/
For a Complete Report
This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report.
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
Last Updated: 5/21/2015
Copyright 2015 National Organization for Rare Disorders, Inc.
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