Susac syndrome

Common Name(s)

Susac syndrome

Susac syndrome is an autoimmune condition that affects very fine blood vessels in the brain, retina, and the cochlea (inner ear) .  It is characterized by three main symptoms: encephalopathy (which can include headache, mild memory losses, personality changes, and confusion); sensorineural hearing loss; and vision loss. Most people do not have all the symptoms of Susac syndrome at the beginning of their illness but develop symptoms over the course of several years. Susac syndrome affects women more than men and the age of onset is usually between 20 and 40 years.  The cause of this condition is still unknown. Corticosteroids, antiplatelets, anticoagulation, and cyclophosphamide have been frequently used to treat Susac syndrome. However, the extent to which individuals respond to treatment is not clear. Improvement may occur spontaneously although in some cases there may be residual dysfunction.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Susac syndrome" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Susac syndrome" returned 24 free, full-text research articles on human participants. First 3 results:

Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study.
 

Author(s): Sven Jarius, Ilka Kleffner, Jan M Dörr, Jaume Sastre-Garriga, Zsolt Illes, Eric Eggenberger, Colin Chalk, Marius Ringelstein, Orhan Aktas, Xavier Montalban, Kai Fechner, Winfried Stöcker, Erich B Ringelstein, Friedemann Paul, Brigitte Wildemann

Journal:

 

Susac syndrome (SuS) is a rare disorder thought to be caused by autoimmune-mediated occlusions of microvessels in the brain, retina and inner ear leading to central nervous system (CNS) dysfunction, visual disturbances due to branch retinal artery occlusions (BRAO), and hearing deficits. ...

Last Updated: 20 Mar 2014

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Reply: Progression of retinal artery occlusion in Susac syndrome.
 

Author(s): Mário Luiz Ribeiro Monteiro

Journal: Arq Bras Oftalmol. ;76(6):391-2.

 

Last Updated: 10 Feb 2014

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Progression of retinal artery occlusion from one eye to the other seems to be a characteristic finding in Susac syndrome.
 

Author(s): Yildiray Yildirim, Taner Kar, Abdullah Kaya

Journal: Arq Bras Oftalmol. ;76(6):391.

 

Last Updated: 10 Feb 2014

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Susac syndrome" returned 3 free, full-text review articles on human participants. First 3 results:

Susac syndrome as an example of a rare vasculopathy.
 

Author(s): Magdalena Olszanecka-Glinianowicz, Antoni Hrycek

Journal: Pol. Arch. Med. Wewn.. ;119(1-2):80-2.

 

Susac syndrome is a rare vasculopathy affecting small vessels of the brain, the retina and the cochlea. The pathogenesis of this syndrome is unknown. It is characterized by a triad of clinical symptoms, i.e. encephalopathy, visual disorders and hearing disorders. The aim of this work ...

Last Updated: 3 Apr 2009

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[Susac syndrome as a cause of sensorineural hearing loss].
 

Author(s): J D Cubillana Herrero, A Soler Valcárcel, I Albaladejo Devis, B Rodríguez González-Herrero, N Minguez Merlos, J A Jiménez Cervantes-Nicolás

Journal: Acta Otorrinolaringol Esp. 2002 May;53(5):379-83.

 

Susac's syndrome is an extremely rare clinical manifestation characterized by the triad of fluctuating sensorineural hearing loss, sudden visual loss and encephalopathy. Probably underdiagnosed, it affects young women who start the clinical history with headache, visual and hearing ...

Last Updated: 20 Aug 2002

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[Small retinal, cochlear, and cerebral infarctions in the young patient, "SICRET" syndrome of Susac syndrome].
 

Author(s): L Mala, M C Bazard, J P Berrod, D Wahl, A Raspiller

Journal: J Fr Ophtalmol. 1998 May;21(5):375-80.

 

A 22-year-old-lady presented with multiple occlusions of the branches of the central retinal artery, accompanied by neuro-encephalic disorders and deafness. This triad is known as SICRET Syndrome (Small Infarction of Cochlear, Retinal and Encephalic Tissue). This rare syndrome, as ...

Last Updated: 19 Nov 1998

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Epidemiological, Clinical and Etiological Features of SUSAC's Syndrome
 

Status: Recruiting

Condition Summary: SUSAC's Syndrome; Encephalopathy; Hearing Loss; Retinal Artery Branch Occlusions

 

Last Updated: 13 Aug 2013

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Investigation of Biomarkers in Susac Syndrome
 

Status: Recruiting

Condition Summary: Susac Syndrome

 

Last Updated: 11 Feb 2013

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