RED-M
MCID: SSC001
MIFTS: 27

Susac Syndrome (RED-M) malady

Rare diseases, Neuronal diseases, Ear diseases, Cardiovascular diseases categories
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Summaries for Susac Syndrome

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NIH Rare Diseases:42 Susac syndrome is an autoimmune condition that affects very fine blood vessels in the brain, retina, and cochlea (inner ear). it is characterized by three main symptoms: encephalopathy (which can cause headache, mild memory loss, 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 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. last updated: 7/21/2014

MalaCards based summary: Susac Syndrome, also known as retinocochleocerebral vasculopathy, is related to retinitis and retinal artery occlusion. Affiliated tissues include brain, retina and eye.

Aliases & Classifications for Susac Syndrome

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Sources:
42NIH Rare Diseases, 48Orphanet, 62UMLS, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet
See all sources

Susac Syndrome, Aliases & Descriptions:

Name: Susac Syndrome 42 48 62
Retinocochleocerebral Vasculopathy 42 48 62
Retinopathy-Encephalopathy-Deafness Associated with Microangiopathy 48
Small Infarctions of Cochlear, Retinal and Encephalic Tissue 48
 
Sicret Syndrome 42
Sicret Syndrome 48
Red-M 48


Classifications:



Characteristics (Orphanet epidemiological data):

48
retinocochleocerebral vasculopathy:
Age of onset: Variable; Age of death: Normal


External Ids:

MESH via Orphanet35 D055955
ICD10 via Orphanet26 I67.7
UMLS via Orphanet63 C2717757

Related Diseases for Susac Syndrome

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Diseases related to Susac Syndrome via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show all 16)
idRelated DiseaseScoreTop Affiliating Genes
1retinitis10.6
2retinal artery occlusion10.5
3central retinal artery occlusion10.4
4multiple sclerosis10.2
5branch retinal artery occlusion10.2
6bipolar disorder10.1
7hepatitis10.1
8multifocal motor neuropathy10.1
9hepatitis c10.1
10sensorineural hearing loss10.1
11cauda equina syndrome10.1
12retinal vasculitis10.1
13cervicitis10.1
14neuropathy10.1
15vasculitis10.1
16endotheliitis10.1

Graphical network of diseases related to Susac Syndrome:



Diseases related to susac syndrome

Symptoms for Susac Syndrome

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Drugs & Therapeutics for Susac Syndrome

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Drug clinical trials:

Search ClinicalTrials for Susac Syndrome

Search NIH Clinical Center for Susac Syndrome

Genetic Tests for Susac Syndrome

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Anatomical Context for Susac Syndrome

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MalaCards organs/tissues related to Susac Syndrome:

32
Brain, Retina, Eye, Spinal cord, Endothelial

Animal Models for Susac Syndrome or affiliated genes

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Publications for Susac Syndrome

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Articles related to Susac Syndrome:

(show top 50)    (show all 76)
idTitleAuthorsYear
1
Update on Susac syndrome: new insights in brain and retinal imaging and treatment options. (24662104)
2014
2
Susac syndrome: diverse clinical findings and treatment. (25295909)
2014
3
Recurrence of Susac Syndrome following 23 Years of Remission. (24987361)
2014
4
Clinical, paraclinical and serological findings in Susac syndrome: an international multicenter study. (24606999)
2014
5
Susac syndrome. (25337735)
2014
6
Unilateral central retinal artery occlusion as the sole presenting sign of Susac syndrome in a young man: case report. (24232952)
2013
7
Susac syndrome: an Italian case. (23828373)
2013
8
Characteristics of Susac syndrome: a review of all reported cases. (23628737)
2013
9
Susac Syndrome Treated with Subcutaneous Immunoglobulin. (24335148)
2013
10
A case of Susac syndrome. (24082778)
2013
11
Progression of retinal artery occlusion from one eye to the other seems to be a characteristic finding in Susac syndrome. (24510092)
2013
12
Unilateral central retinal artery occlusion as the sole presenting sign of Susac syndrome in a young man: case report. (23929083)
2013
13
A case of Susac syndrome with cervical spinal cord involvement on MRI. (24365248)
2013
14
Susac syndrome in a patient with human immunodeficiency virus infection. (23690260)
2013
15
Reply: Progression of retinal artery occlusion in Susac syndrome. (24510093)
2013
16
Reply: Fibrin-like material in the retinal arterioles and fluorescein angiography findings in Susac syndrome. (24232953)
2013
17
Susac syndrome. A differential diagnosis for demyelination. (23291802)
2013
18
Susac syndrome: clinical characteristics and treatment in 29 new cases. (22221557)
2012
19
A brief review of Susac syndrome. (22640902)
2012
20
Exacerbation of Susac syndrome retinopathy by interferon Beta-1a. (22801853)
2012
21
Susac syndrome and pregnancy: disease management. (21325959)
2011
22
Recurrent bilateral branch retinal artery occlusion with hearing loss and encephalopathy: the first case report of Susac syndrome in Korea. (22065912)
2011
23
Review of Susac syndrome. (21897238)
2011
24
Susac syndrome: outcome of unilateral cochlear implantation. (21481296)
2011
25
Gass plaques and fluorescein leakage in Susac Syndrome. (20880549)
2010
26
CT and MR imaging of Susac syndrome in a young male presenting with acute disorientation. (20189079)
2010
27
Susac syndrome. (20686320)
2010
28
Severe retention hyperkeratosis occurring with Susac syndrome. (21062602)
2010
29
Susac syndrome as an example of a rare vasculopathy. (19341183)
2009
30
Case 142: Susac syndrome. (19188329)
2009
31
Is Susac syndrome (microangiopathy of the inner ear, retina, and central nervous system) an underdiagnosed cause of sensorineural hearing loss? (18633920)
2008
32
Diffusion tensor imaging demonstrates fiber impairment in Susac syndrome. (17959768)
2008
33
Susac syndrome and vestibular-evoked myogenic potentials. (18359373)
2008
34
Susac syndrome in a patient with hepatitis C. (17414876)
2007
35
Long-term outcome in Susac syndrome. (17435589)
2007
36
Re: Susac syndrome in four male patients. (18040266)
2007
37
Susac syndrome in a young child. (17476496)
2007
38
Susac syndrome in four male patients. (17151493)
2006
39
Susac syndrome with frontal intermittent rhythmic delta activity (FIRDA). (17168166)
2006
40
Susac syndrome: retinocochleocerebral vasculopathy. (15185430)
2004
41
Susac syndrome: serial diffusion-weighted MR imaging. (15607101)
2004
42
Evolution of lesions in Susac syndrome at serial MR imaging with diffusion-weighted imaging and apparent diffusion coefficient values. (15140708)
2004
43
Susac syndrome. (12439681)
2002
44
Susac syndrome and multiple sclerosis. (11296064)
2001
45
Susac syndrome: a vasospastic disorder? (11456021)
2001
46
A case of Susac syndrome. (11057732)
2000
47
Susac syndrome. (10844072)
2000
48
Microangiopathy of the inner ear, retina, and brain (Susac syndrome): report of a case. (10628717)
2000
49
Susac syndrome: microangiopathy of the retina, cochlea and brain. (11097286)
2000
50
Microangiopathy of the brain, retina, and cochlea (Susac syndrome). A report of five cases and a review of the literature. (9627654)
1998

Variations for Susac Syndrome

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Expression for genes affiliated with Susac Syndrome

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Search GEO for disease gene expression data for Susac Syndrome.

Pathways for genes affiliated with Susac Syndrome

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Compounds for genes affiliated with Susac Syndrome

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GO Terms for genes affiliated with Susac Syndrome

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Products for genes affiliated with Susac Syndrome

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  • Antibodies
  • Proteins
  • Lysates

Sources for Susac Syndrome

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet