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:

Search ClinicalTrials for Subacute Sclerosing Panencephalitis

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, T cells, Cortex, 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
PD-1 gene polymorphism in children with subacute sclerosing panencephalitis. (23504559)
2013
2
Epilepsy in children with subacute sclerosing panencephalitis. (24073547)
2013
3
Subacute sclerosing panencephalitis with bilateral inferior collicular hyperintensity on magnetic resonance imaging brain. (23349608)
2012
4
Fulminant subacute sclerosing panencephalitis in an individual with a perinatally acquired human immunodeficiency virus infection. (22944871)
2012
5
Current therapies and future perspectives in subacute sclerosing panencephalitis. (22449219)
2012
6
Acquired immunodeficiency syndrome with subacute sclerosing panencephalitis. (23044024)
2012
7
Status dystonicus and rhabdomyolysis in a patient with subacute sclerosing panencephalitis. (22397054)
2012
8
Ocular findings in subacute sclerosing panencephalitis. (21428756)
2011
9
Subacute sclerosing panencephalitis presenting as mania. (21808475)
2011
10
Subacute sclerosing panencephalitis in a tertiary care centre in post measles vaccination era. (22010482)
2009
11
Progressive cognitive decline and myoclonus in a young woman: clinicopathological conference at the Edinburgh Advanced Neurology Course, 2007. Variant CJD. Subacute sclerosing panencephalitis. (18796584)
2008
12
Two cases of subacute sclerosing panencephalitis associated with brainstem involvement. (17496325)
2007
13
Full-length sequence analysis of subacute sclerosing panencephalitis (SSPE) virus, a mutant of measles virus, isolated from brain tissues of a patient shortly after onset of SSPE. (16858143)
2006
14
Subacute sclerosing panencephalitis--the continuing threat. (17243579)
2006
15
Inhibition of measles virus and subacute sclerosing panencephalitis virus by RNA interference. (16530274)
2006
16
Adult onset subacute sclerosing panencephalitis: clinical profile of 39 patients from a tertiary care centre. (16464898)
2006
17
Gene expression profiles in peripheral blood mononuclear cells from patients with subacute sclerosing panencephalitis using oligonucleotide microarrays. (16036810)
2005
18
Pharmacokinetics and effects of ribavirin following intraventricular administration for treatment of subacute sclerosing panencephalitis. (15561836)
2004
19
Subacute sclerosing panencephalitis after intrauterine infection. (15384893)
2004
20
Fulminating adult-onset subacute sclerosing panencephalitis in a 49-year-old man. (12925376)
2003
21
Trial of intraventricular ribavirin and interferon-alpha combination therapy for subacute sclerosing panencephalitis (SSPE) in Japan]. (12875209)
2003
22
Combination therapy with intraventricular interferon-alpha and ribavirin for subacute sclerosing panencephalitis and monitoring measles virus RNA by quantitative PCR assay. (12850518)
2003
23
A case of subacute sclerosing panencephalitis preceded by epileptic seizures: evolutional EEG changes. (12767461)
2003
24
Viral studies in the cerebrospinal fluid in subacute sclerosing panencephalitis. (12099745)
2002
25
Interleukin-1beta, interleukin-1 receptor antagonist levels in patients with subacute sclerosing panencephalitis and the effects of different treatment protocols. (11417607)
2001
26
Hemadsorption expressed by cloned H genes from subacute sclerosing panencephalitis (SSPE) viruses and their possible progenitor measles viruses isolated in Osaka, Japan. (11270608)
2001
27
Cloning the antibody response in humans with chronic inflammatory disease: immunopanning of subacute sclerosing panencephalitis (SSPE) brain sections with antibody phage libraries prepared from SSPE brain enriches for antibody recognizing measles virus antigens in situ. (10627565)
2000
28
Measles virus-specific T helper 1/T helper 2-cytokine production in subacute sclerosing panencephalitis. (10822325)
2000
29
Cloning the antibody response in humans with inflammatory central nervous system disease: analysis of the expressed IgG repertoire in subacute sclerosing panencephalitis brain reveals disease-relevant antibodies that recognize specific measles virus antigens. (10477623)
1999
30
Nucleotide sequences of the matrix protein gene of subacute sclerosing panencephalitis viruses compared with local contemporary isolates from patients with acute measles. (9660076)
1998
31
The matrix gene expression of subacute sclerosing panencephalitis (SSPE) virus (Osaka-1 strain): a comparison of two sibling viruses isolated from different lobes of an SSPE brain. (9886150)
1998
32
Long-term follow-up of patients with subacute sclerosing panencephalitis treated with intraventricular alpha-interferon. (9040751)
1997
33
Increased levels of interleukin-1beta and soluble intercellular adhesion molecule-1 in cerebrospinal fluid of patients with subacute sclerosing panencephalitis. (9041345)
1997
34
Treatment of subacute sclerosing panencephalitis. (9430310)
1997
35
Healthy infant delivered by a mother with subacute sclerosing panencephalitis during pregnancy. (8960759)
1996
36
Major histocompatibility complex class I expression on neurons in subacute sclerosing panencephalitis and experimental subacute measles encephalitis. (8786403)
1996
37
Long-term survival and temporary clinical improvement in subacute sclerosing panencephalitis. (8858710)
1996
38
Subacute sclerosing panencephalitis: evaluation with CT and MR. (8730198)
1996
39
Periodic electroencephalographic pattern in subacute sclerosing panencephalitis modified by preexisting damaged cerebral hemisphere. (9020803)
1996
40
Atypical clinical and electroencephalographic pattern in a patient with subacute sclerosing panencephalitis. (7487538)
1995
41
Association of measles virus with neurofibrillary tangles in subacute sclerosing panencephalitis: a combined in situ hybridization and immunocytochemical investigation. (8072641)
1994
42
Generation and properties of measles virus mutations typically associated with subacute sclerosing panencephalitis. (8030963)
1994
43
Defective measles virus in human subacute sclerosing panencephalitis brain. (8030228)
1994
44
Subacute sclerosing panencephalitis in Wales. (8140214)
1994
45
Functional analysis of matrix proteins expressed from cloned genes of measles virus variants that cause subacute sclerosing panencephalitis reveals a common defect in nucleocapsid binding. (8445713)
1993
46
Complete nucleotide sequence of the phosphoprotein of the Yamagata-1 strain of a defective subacute sclerosing panencephalitis (SSPE) virus. (1536889)
1992
47
The distribution of Alzheimer's neurofibrillary tangles and gliosis in chronic subacute sclerosing panencephalitis. (1698006)
1990
48
Subacute sclerosing panencephalitis, progressive rubella panencephalitis, and multifocal leukoencephalopathy. (2183315)
1990
49
Oral ribavirin therapy for subacute sclerosing panencephalitis. (2926165)
1989
50
A study of subacute sclerosing panencephalitis in general practice. (3693931)
1987

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

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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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