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Susac Syndrome

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A rare disorder consisting of microangiopathy of brain, retina, and inner ear ARTERIOLES. It is characterized by the clinical triad of encephalopathy, BRANCH RETINAL ARTERY OCCLUSION and VERTIGO/hearing loss.

Also Known As:

Retinocochleocerebral Vasculopathy; Retinocochleocerebral Vasculopathies; Susacs Syndrome; Syndrome, Susac; Syndrome, Susac's; Vasculopathies, Retinocochleocerebral; Vasculopathy, Retinocochleocerebral; Susac's Syndrome

Networked: 60 relevant articles (3 outcomes, 3 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Necrosis
2.	Retinal Artery Occlusion
3.	Multiple Sclerosis
4.	Hearing Loss (Hearing Impairment)
5.	Leukoencephalopathies

Experts

1.	Kleffner, Ilka: 5 articles (08/2015 - 01/2012)
2.	Dörr, Jan: 5 articles (08/2015 - 01/2012)
3.	Susac, John O: 5 articles (12/2011 - 04/2003)
4.	Duning, Thomas: 4 articles (08/2015 - 06/2013)
5.	Ringelstein, Marius: 4 articles (08/2015 - 01/2012)
6.	Aktas, Orhan: 3 articles (08/2015 - 01/2012)
7.	Paul, Friedemann: 3 articles (08/2015 - 01/2012)
8.	Lubow, Martin: 3 articles (12/2011 - 10/2008)
9.	Hartung, Hans-Peter: 2 articles (08/2015 - 01/2012)
10.	Jarius, Sven: 2 articles (08/2015 - 06/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Susac Syndrome:

1.	Retinaldehyde (Retinal)IBA 08/18/2015 - "Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography." 01/01/2014 - "Update on Susac syndrome: new insights in brain and retinal imaging and treatment options." 01/01/2014 - "The occurrence of the characteristic retinal pathology with small artery occlusions and a segmental vasculopathy should always lead to the differential diagnosis of Susac syndrome." 01/01/2014 - "Three patients with confirmed Susac syndrome were evaluated for disease-specific retinal pathologies. " 01/01/2014 - "We report an interesting case of asymptomatic retinal involvement in an encephalopathic patient enabling early identification of Susac's syndrome. "
2.	Fluorescein (Funduscein)FDA LinkGeneric 01/01/2014 - "We confirm that OCT is a useful diagnostic tool in Susac's syndrome and helps to differentiate it from MS. Furthermore, OCT may be a non-invasive alternative to fluorescein angiography in longitudinal follow up of these patients. " 01/01/2014 - "Susac's syndrome: the value of fundus fluorescein angiography." 12/15/2010 - "Gass plaques and fluorescein leakage in Susac Syndrome." 01/01/2012 - "Retinal fluorescein angiography helped the diagnosis of Susac's syndrome." 07/01/2004 - "The objective of this study was to describe the clinical manifestations; radiographic, audiometric, and retinal fluorescein angiography findings; pathogenesis and treatment of Susac syndrome with review of the literature. "
3.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 09/01/2000 - "[Susac's syndrome: improvement with combined cyclophosphamide and intravenous immunoglobulin therapy]." 06/01/2015 - "Cyclophosphamide and methotrexate in Susac's Syndrome: a successful sequential therapy in a case with involvement of the cerebellum." 05/01/2013 - "The patient was diagnosed with Susac syndrome and treated with methylprednisolone and cyclophosphamide, resulting in slight improvement and stabilization. "
4.	infliximab (Remicade)FDA Link 03/15/2011 - "We report a case of Susac's syndrome in which the patient improved immediately after tumour necrosis factor (TNF) inhibition with the monoclonal antibody, infliximab."
5.	OxygenIBA 12/01/1996 - "This is the first report of hyperbaric oxygen treatment for Susac syndrome. " 12/01/1996 - "Reversal of visual loss with hyperbaric oxygen treatment in a patient with Susac syndrome." 12/01/1996 - "The purpose of the study is to report on a 34-year-old woman with Susac syndrome who presented on two separate occasions with visual acuity loss from a recurrent branch retinal artery occlusion and underwent hyperbaric oxygen treatment with favorable outcome. "
6.	AntibodiesIBA 12/01/2011 - "Susac syndrome: an organ-specific autoimmune endotheliopathy syndrome associated with anti-endothelial cell antibodies." 10/15/2009 - "Anti-endothelial serum antibodies in a patient with Susac's syndrome." 06/15/2014 - "Susac's syndrome (SS) is an infrequent neurological disorder characterized by the clinical triad of encephalopathy, branch retinal artery occlusion and hearing loss due to an autoimmune endotheliopathy associated with anti-endothelial cell antibodies. " 12/21/2006 - "Treatment with IVIg and corticosteroids provides a therapeutic option that avoids the toxicities of chemotherapy and suggests the possible importance of pathologic antibodies in the pathogenesis of Susac syndrome."
7.	Methylprednisolone FDA LinkGeneric 05/01/2013 - "The patient was diagnosed with Susac syndrome and treated with methylprednisolone and cyclophosphamide, resulting in slight improvement and stabilization. " 11/01/2006 - "A diagnosis of Susac syndrome was made and treatment with methylprednisolone, ASA, and Nimodipine was instituted. " 01/01/2006 - "A combination of intravenous methylprednisolone and immunoglobulins, recommended for the treatment of Susac syndrome, was administered. "
8.	Indocyanine Green (Cardio-Green)FDA LinkGeneric 04/01/2012 - "Indocyanine-green angiography findings in Susac's syndrome." 10/01/2008 - "Fluorescein and indocyanine green angiographies in Susac syndrome." 11/01/2007 - "Fluorescein and indocyanine green angiographies in Susac syndrome."
9.	Immunoglobulins (Immunoglobulin)IBA 01/01/2014 - "Susac syndrome treated with subcutaneous immunoglobulin." 01/01/2006 - "A combination of intravenous methylprednisolone and immunoglobulins, recommended for the treatment of Susac syndrome, was administered. " 01/01/2014 - "We present the case histories of 2 young women with Susac syndrome presenting with several episodes of encephalopathy, branch retinal artery occlusions, and hearing loss that were treated with different immunosuppressive drugs, glucocorticoids and intravenous immunoglobulin. "
10.	Adrenal Cortex Hormones (Corticosteroids)IBA 12/01/2009 - "A diagnosis of retino-cochlear-cerebral vasculopathy or Susac's syndrome was established and steroid-based immunotherapy with high-dose corticosteroids was initiated. " 12/21/2006 - "We describe two patients with Susac syndrome and their response to intravenous immune globulin (IVIg) and corticosteroids. " 12/21/2006 - "Treatment of Susac syndrome with gamma globulin and corticosteroids." 12/21/2006 - "Treatment with IVIg and corticosteroids provides a therapeutic option that avoids the toxicities of chemotherapy and suggests the possible importance of pathologic antibodies in the pathogenesis of Susac syndrome."

Therapies and Procedures

1.	Cochlear Implantation 12/01/2012 - "Successful cochlear implantation in a Susac syndrome patient." 10/01/2011 - "To report the audiological outcomes of cochlear implantation (CI) in a young woman with Susac syndrome after a 6-month follow-up period. " 08/01/2011 - "In this Susac syndrome patient, the outcome of cochlear implantation was encouraging, notwithstanding the possible involvement of retrocochlear pathways." 08/01/2011 - "Susac syndrome: outcome of unilateral cochlear implantation." 01/01/2009 - "We present a unique case of bilateral cochlear implantation in a Susac syndrome patient and seek to describe in detail the specific nature of the otologic manifestations of this disease. "
2.	Immunotherapy 12/01/2009 - "[Aggressive immunotherapy in Susac's syndrome]." 12/01/2009 - "A diagnosis of retino-cochlear-cerebral vasculopathy or Susac's syndrome was established and steroid-based immunotherapy with high-dose corticosteroids was initiated. "
3.	Drug Therapy (Chemotherapy) 12/21/2006 - "Treatment with IVIg and corticosteroids provides a therapeutic option that avoids the toxicities of chemotherapy and suggests the possible importance of pathologic antibodies in the pathogenesis of Susac syndrome."
4.	Cochlear Implants (Cochlear Implant) 10/01/2011 - "The cochlear implant was beneficial as an attempt to restore hearing and improve communication abilities in this patient with profound sensorineural hearing loss secondary to Susac syndrome. "