Susac's Syndrome / Disease

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Susac's Syndrome

Synonyms of Susac's Syndrome

Susac syndrome

Disorder Subdivisions

General Discussion
Susac?s syndrome is a rare disorder characterized by impaired brain function (encephalopathy), blockage (occlusion) of the arteries that supply blood to the retina (branched retinal arterial occlusion [BRAO]), and hearing loss. Two main forms of Susac?s syndrome have been identified. In one form, encephalopathy is the main finding; in the other form, BRAO and hearing loss occur without signs of brain disease. The specific symptoms and severity of Susac?s syndrome vary from one person to another. The encephalopathic form of Susac?s syndrome often improves spontaneously even without treatment (self-limited); the other form is frequently a chronic disorder. Although considered rare, Susac?s syndrome is being recognized more often worldwide and its true frequency in the general population is unknown.

Susac?s syndrome is considered an autoimmune endotheliopathy. An autoimmune disorder occurs when the body?s own immune system mistakenly attacks healthy tissue. An endotheliopathy is any disorder that primarily involves the endothelium, which is the thin layer of cells that line the walls of blood vessels. In Susac?s syndrome, the smallest blood vessels (microvasculature) of the circulatory system such as the capillaries, venules and arterioles are affected. Specifically, the microvasculature of the brain, retinas and inner ears are involved.

Organizations related to Susac's Syndrome

American Academy of Audiology
11730 Plaza America
Reston VA 20190
Phone #: 703-790-8466
800 #: 800-222-2336
e-mail: [email protected]
Home page: http://www.audiology.org
American Autoimmune Related Diseases Association, Inc.
22100 Gratiot Avenue
Eastpointe MI 48021
Phone #: 586-776-3900
800 #: 800-598-4668
e-mail: [email protected]
Home page: http://www.aarda.org/
AutoImmunity Community
None
None None None
Phone #: (91-9) -552-9057
800 #: N/A
e-mail: [email protected]
Home page: http://autoimmunitycommunity.org
Better Hearing Institute
515 King Street, Suite 420
Alexandria VA 22314
Phone #: 703-684-3391
800 #: 800-327-9355
e-mail: [email protected]
Home page: http://www.betterhearing.org
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg MD 20898-8126
Phone #: 301-519-3194
800 #: 888-205-2311
e-mail: [email protected]
Home page: http://www.genome.gov/10000409
National Institute of Neurological Disorders and Stroke (NINDS)
31 Center Drive
Bethesda MD 20892-2540
Phone #: 301-496-5751
800 #: 800-352-9424
e-mail: [email protected]
Home page: http://www.ninds.nih.gov/

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc. ? (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html