SSPE
MCID: SBC001
MIFTS: 53

Subacute Sclerosing Panencephalitis (SSPE) malady

Infectious diseases, Immune diseases categories

Summaries for Subacute Sclerosing Panencephalitis

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8Disease Ontology, 43NINDS, 63Wikipedia, 46OMIM, 32MalaCards
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NINDS:43 Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of children and young adults that affects the central nervous system (CNS). It is a slow, but persistent, viral infection caused by defective measles virus. SSPE has been reported from all parts of the world, but it is considered a rare disease in developed countries, with fewer than 10 cases per year reported in the United States. The incidence of SSPE declined by at least 90 percent in countries that have practiced widespread immunization with measles vaccine. The incidence of SSPE is still high in developing countries such as India and Eastern Europe. There is a higher incidence among males than females (male/female: 3/1). Most youngsters with SSPE have a history of measles infection at an early age, usually younger than 2 years, followed by a latent period of 6 to 8 years before neurological symptoms begin. Despite the long interval between the measles infection and the onset of SSPE, researchers think that the infection of the brain occurs soon after the primary bout with measles and progresses slowly. Why it persists and progresses still isn't clear. The initial symptoms of SSPE are subtle and include mild mental deterioration (such as memory loss) and changes in behavior (such as irritability) followed by disturbances in motor function, including uncontrollable involuntary jerking movements of the head, trunk or limbs called myoclonic jerks. Seizures may also occur. Some people may become blind. In advanced stages of the disease, individuals may lose the ability to walk, as their muscles stiffen or spasm. There is progressive deterioration to a comatose state, and then to a persistent vegetative state. Death is usually the result of fever, heart failure, or the brain's inability to continue controlling the autonomic nervous system.

MalaCards: Subacute Sclerosing Panencephalitis, also known as van bogaert's sclerosing leukoencephalitis, is related to measles and encephalitis. An important gene associated with Subacute Sclerosing Panencephalitis is MX1 (myxovirus (influenza virus) resistance 1, interferon-inducible protein p78 (mouse)), and among its related pathways are Toll Comparative Pathway and Immune response IFN alpha/beta signaling pathway. The compounds rebif and ribavirin have been mentioned in the context of this disorder. Affiliated tissues include brain, brain and heart, and related mouse phenotypes are hematopoietic system and immune system.

Disease Ontology:8 A viral infectious disease that results in infection located in brain, has material basis in measles virus which is immune resistant (which can be a result of a mutation of the virus itself).

Wikipedia:63 Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis that affects... more...

Description from OMIM:46 260470

Aliases & Classifications for Subacute Sclerosing Panencephalitis

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8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 10DISEASES, 44Novoseek, 60UMLS, 27ICD9CM, 56SNOMED-CT, 34MeSH, 46OMIM, 39NCIt, 25ICD10
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Infectious diseases
Anatomical: Immune diseases


Aliases & Descriptions:

subacute sclerosing panencephalitis 8 42 43 10 44 60
van bogaert's sclerosing leukoencephalitis 8
subacute sclerosing leukoencephalopathy 8
immunosuppressive measles encephalitis 8
sspe 42


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Disease Ontology8 DOID:8970
ICD9CM27 046.2
MeSH34 D013344
OMIM46 260470
NCIt39 C85171
ICD1025 A81.1

Related Diseases for Subacute Sclerosing Panencephalitis

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Subacute Sclerosing Panencephalitis:



Diseases related to subacute sclerosing panencephalitis

Clinical Features for Subacute Sclerosing Panencephalitis

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46OMIM
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Clinical features from OMIM:

260470

Drugs & Therapeutics for Subacute Sclerosing Panencephalitis

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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Search NIH Clinical Center for Subacute Sclerosing Panencephalitis

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Genetic Tests for Subacute Sclerosing Panencephalitis

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Anatomical Context for Subacute Sclerosing Panencephalitis

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32MalaCards, 14FMA
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MalaCards organs/tissues related to Subacute Sclerosing Panencephalitis:

32
Brain, Heart, Cortex, T cells, Endothelial

FMA organs/tissues related to Subacute Sclerosing Panencephalitis:

14
Brain

Animal Models for Subacute Sclerosing Panencephalitis or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Subacute Sclerosing Panencephalitis:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053979.9IFNGR1, IRF1, LIF, MAPT, CXCL10, IL12RB1
2MP:00053879.5SLAMF1, MOG, TLR3, IFIH1, IFNGR1, IRF1

Publications for Subacute Sclerosing Panencephalitis

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50PubMed
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Articles related to Subacute Sclerosing Panencephalitis:

(show top 50)    (show all 500)
idTitleAuthorsYear
1
Atypical subacute sclerosing panencephalitis with short onset latency. (24222287)
2013
2
Atypical subacute sclerosing panencephalitis with short onset latency. (23474932)
2013
3
Subacute sclerosing panencephalitis and chronic viral encephalitis. (23622327)
2013
4
Total macular atrophy in subacute sclerosing panencephalitis. (22496020)
2012
5
Nonconvulsive status epilepticus on electroencephalography: an atypical presentation of subacute sclerosing panencephalitis in two children. (23094174)
2012
6
Subacute sclerosing panencephalitis in immunized Thai children. (22619930)
2011
7
Prognosis in subacute sclerosing panencephalitis. (21745204)
2011
8
Drug-indiced aseptic meningitis: development of subacute sclerosing panencephalitis following repeated intraventricular infusion therapy with interferon alpha/beta. (21748242)
2011
9
Interferon production by cells infected with subacute sclerosing panencephalitis (SSPE) virus or measles virus. (21967871)
2011
10
A 22-year-old Australian woman with atypical subacute sclerosing panencephalitis diagnosed at postmortem. (20605468)
2010
11
Serial diffusion-weighted imaging in subacute sclerosing panencephalitis. (18486827)
2008
12
Clinical profile of subacute sclerosing panencephalitis. (18798579)
2008
13
Continuous intrathecal interferon alpha application in subacute sclerosing panencephalitis. (17721393)
2007
14
Subacute sclerosing panencephalitis: is there something different in the younger children? (16829007)
2006
15
Elevated interleukin-12 and CXCL10 in subacute sclerosing panencephalitis. (16226466)
2005
16
Alterations in cell-mediated immune response in subacute sclerosing panencephalitis. (16223530)
2005
17
Antibodies to borna disease virus in subacute sclerosing panencephalitis. (16148853)
2005
18
Unilateral chorioretinitis: an initial manifestation of subacute sclerosing panencephalitis. (16459543)
2005
19
Subacute sclerosing panencephalitis: results of the Canadian Paediatric Surveillance Program and review of the literature. (16356180)
2005
20
Retrospective evaluation of interferon-beta treatment in subacute sclerosing panencephalitis. (15639700)
2004
21
Subacute sclerosing panencephalitis with fulminating course: follow-up magnetic resonance spectroscopy (MRS) findings. (15119488)
2004
22
The epidemiology of subacute sclerosing panencephalitis in England and Wales 1990-2002. (15557053)
2004
23
Subacute sclerosing panencephalitis in children: prevalence in South China. (15246492)
2004
24
Wild-type measles virus in brain tissue of children with subacute sclerosing panencephalitis, Argentina. (14609476)
2003
25
Neurodegenerative mechanisms in subacute sclerosing panencephalitis. (12546425)
2002
26
Subacute sclerosing panencephalitis. (11807185)
2002
27
Seizures in a boy with subacute sclerosing panencephalitis during high-dose intrathecal interferon-alpha therapy. (12160981)
2002
28
Spatial and temporal disease progression of adult-onset subacute sclerosing panencephalitis. (12034805)
2002
29
Evidence that the hypermutated M protein of a subacute sclerosing panencephalitis measles virus actively contributes to the chronic progressive CNS disease. (11878891)
2001
30
Implication of sensorimotor integration in the generation of periodic dystonic myoclonus in subacute sclerosing panencephalitis (SSPE). (11104202)
2000
31
Subacute sclerosing panencephalitis : CT and MR imaging in a rapidly progressive case. (10625904)
1999
32
Subacute sclerosing panencephalitis presenting as acute disseminated encephalomyelitis: a case report. (9077490)
1997
33
Measles, mumps, rubella vaccine induced subacute sclerosing panencephalitis. (9567594)
1997
34
Mental assessment in subacute sclerosing panencephalitis: Hacettepe Cognitive Short Assessment Scale. (9309525)
1997
35
Can cytotoxic drugs cure subacute sclerosing panencephalitis? (7623548)
1995
36
Chronological SPECT studies of a patient with subacute sclerosing panencephalitis. (8214334)
1993
37
Effect of treatment on oligoclonal IgG bands and intrathecal IgG synthesis in sequential cerebrospinal fluid and serum from patients with subacute sclerosing panencephalitis. (1381411)
1992
38
Subacute sclerosing panencephalitis virus dominantly interferes with replication of wild-type measles virus in a mixed infection: implication for viral persistence. (1548746)
1992
39
Subacute sclerosing panencephalitis presenting as cortical blindness. (1604738)
1992
40
Role of biased hypermutation in evolution of subacute sclerosing panencephalitis virus from progenitor acute measles virus. (2016756)
1991
41
Subacute sclerosing panencephalitis presenting as simple partial seizures. (2045631)
1991
42
Molecular analysis of structural protein genes of the Yamagata-1 strain of defective subacute sclerosing panencephalitis virus. I. Nucleotide sequence of the nucleoprotein gene. (1698325)
1990
43
Administration of human leukocyte interferon to patients with subacute sclerosing panencephalitis. (2817295)
1989
44
Incidence of subacute sclerosing panencephalitis following measles and measles vaccination in Japan. (2807674)
1989
45
Cerebral glucose metabolism in the course of subacute sclerosing panencephalitis. (2783367)
1989
46
Multiple viral mutations rather than host factors cause defective measles virus gene expression in a subacute sclerosing panencephalitis cell line. (3346948)
1988
47
HLA and C4 in subacute sclerosing panencephalitis. (3672494)
1987
48
Subacute sclerosing panencephalitis: detection of measles antibody in serum and cerebrospinal fluid by enzyme-linked immunosorbent assay, complement fixation and hemagglutination inhibition. (3574144)
1987
49
Long-term intramuscular recombinant DNA interferon alpha 2 therapy in subacute sclerosing panencephalitis: reduction of serum measles antibodies without clinical improvement. (3622585)
1987
50
Rapid degradation restricts measles virus matrix protein expression in a subacute sclerosing panencephalitis cell line. (3464005)
1986

Genetic Variations for Subacute Sclerosing Panencephalitis

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Expression for genes affiliated with Subacute Sclerosing Panencephalitis

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Subacute Sclerosing Panencephalitis

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Pathways for genes affiliated with Subacute Sclerosing Panencephalitis

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51QIAGEN, 12EMD Millipore, 53Reactome, 4Cell Signaling Technology, 37NCBI BioSystems Database, 52R&D Systems, 29KEGG
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Pathways related to Subacute Sclerosing Panencephalitis according to GeneCards/GeneDecks:

(show all 12)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.3IRF1, IFNGR1, TLR3
2
Immune response IFN alpha/beta signaling pathway
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10.3MX1, IRF1, IFNA1
310.3IRF1, IFIH1, TLR3
410.3CXCL10, IRF1, IFNGR1
5
Hide members
10.3TLR3, IFNGR1, IL12RB1
6
Hide members
10.2IFNGR1, IFNA1, IRF1, MX1
710.2IL12RB1, LIF, IFNA1, IFNGR1
8
Hide members
10.1TLR3, IFIH1, IFNGR1, IFNA1, CXCL10
9
Hide members
10.1CCL16, TLR3, LIF, CXCL10, IL12RB1
10
Hide members
10.0CCL16, IFNGR1, IFNA1, LIF, CXCL10, IL12RB1
11
Hide members
10.0TLR3, IFIH1, IFIH1, IFNGR1, IFNA1, IRF1
12
Hide members
9.9SLAMF1, TLR3, IFIH1, IFNGR1, IFNA1, CD46

Compounds for genes affiliated with Subacute Sclerosing Panencephalitis

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44Novoseek, 49PharmGKB, 11DrugBank, 28IUPHAR, 59Tocris Bioscience, 24HMDB, 2BitterDB
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Compounds related to Subacute Sclerosing Panencephalitis according to GeneCards/GeneDecks:

(show all 24)
idCompoundScoreTop Affiliating Genes
1rebif4410.6MX1, IFNA1
2ribavirin44 49 1112.6MX1
3avonex4410.6MX1, IFNA1
4imiquimod44 28 59 1113.6TLR3, IFNA1, MX1
5betaseron4410.6MX1, IFNA1
6inosine monophosphate4410.5TLR3, IFNA1, IRF1
72,5-oligoadenylate4410.5TLR3, IFNA1, IRF1, CXCL10, MX1
8poly(i-c)4410.5TLR3, IFNA1, IRF1, CXCL10, MX1
9rantes4410.5CD46, LIF, IRF1, IFNA1, CCL16
10polyinosinic-polycytidylic acid4410.5TLR3, IFIH1, IFNA1, IRF1, CXCL10
11cycloheximide4410.5IFNA1, IRF1, MAPT, CXCL10, MX1
12dexamethasone44 49 28 1113.4TLR3, IFNA1, LIF, CD46
13ifn-alpha4410.4TLR3, IFNA1, IRF1, LIF, CXCL10, MX1
14resiquimod44 5911.4IFNA1, TLR3
15neopterin4410.4MX1, CXCL10, IFNA1
16ccl344 2811.4CCL16, IFNA1, CXCL10
17nitric oxide44 11 2412.3MOG, TLR3, IRF1, LIF, CD46, CXCL10
18retinoic acid44 2411.3TLR3, IFIH1, IFNA1, IRF1, LIF, MAPT
19serine4410.3MOG, TLR3, GNLY, IRF1, LIF, MAPT
20chloroquine44 2 49 28 1114.3IFNA1, MAPT, CXCL10
21actinomycin d4410.2MAPT, LIF, IRF1, IFNA1
22tyrosine4410.2TLR3, IFNGR1, IFNA1, IRF1, LIF, CD46
23gp 1304410.2IL12RB1, LIF, IFNGR1
24glatiramer acetate44 1111.0MOG, IFNA1

GO Terms for genes affiliated with Subacute Sclerosing Panencephalitis

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16Gene Ontology
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Biological processes related to Subacute Sclerosing Panencephalitis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1detection of virusGO:00959710.4IFIH1, TLR3
2positive regulation of interferon-beta productionGO:03272810.4IRF1, IFIH1, TLR3
3response to virusGO:00961510.3MX1, IFNGR1, IFIH1
4defense response to virusGO:05160710.2TLR3, IRF1, CXCL10, MX1
5cytokine-mediated signaling pathwayGO:01922110.1IFNGR1, IRF1, IL12RB1, MX1
6signal transductionGO:0071659.8TLR3, IFNGR1, IGSF1, CXCL10, IL12RB1, MX1

Products for genes affiliated with Subacute Sclerosing Panencephalitis

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Sources for Subacute Sclerosing Panencephalitis

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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
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
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