SSPE
MCID: SBC001
MIFTS: 51

Subacute Sclerosing Panencephalitis (SSPE) malady

Rare diseases, Infectious diseases, Immune diseases, Neuronal diseases categories
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Summaries for Subacute Sclerosing Panencephalitis

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8Disease Ontology, 44NINDS, 65Wikipedia, 47OMIM, 33MalaCards
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NINDS:44 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 Immune response IFN alpha beta signaling pathway and Influenza A. The compounds 2,5-oligoadenylate and poly(i-c) have been mentioned in the context of this disorder. Affiliated tissues include brain, brain and heart, and related mouse phenotypes are immune system and hematopoietic 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:65 Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis that affects... more...

Description from OMIM:47 260470

Aliases & Classifications for Subacute Sclerosing Panencephalitis

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8Disease Ontology, 43NIH Rare Diseases, 44NINDS, 10DISEASES, 45Novoseek, 62UMLS, 40NCIt, 58SNOMED-CT, 27ICD9CM, 47OMIM, 35MeSH, 25ICD10
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Classifications:



Aliases & Descriptions:

subacute sclerosing panencephalitis 8 43 44 10 45 62
van bogaert's sclerosing leukoencephalitis 8
subacute sclerosing leukoencephalopathy 8
immunosuppressive measles encephalitis 8
sspe 43


External Ids:

Disease Ontology8 DOID:8970
NCIt40 C85171
ICD9CM27 046.2
OMIM47 260470
MeSH35 D013344
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

Symptoms for Subacute Sclerosing Panencephalitis

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47OMIM
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Clinical features from OMIM:

260470

Drugs & Therapeutics for Subacute Sclerosing Panencephalitis

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42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Subacute Sclerosing Panencephalitis

Search NIH Clinical Center for Subacute Sclerosing Panencephalitis

Genetic Tests for Subacute Sclerosing Panencephalitis

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Anatomical Context for Subacute Sclerosing Panencephalitis

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33MalaCards, 14FMA
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MalaCards organs/tissues related to Subacute Sclerosing Panencephalitis:

33
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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37MGI
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MGI Mouse Phenotypes related to Subacute Sclerosing Panencephalitis:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053877.7IRF1, MOG, MAPT, BTLA, TNFRSF14
2MP:00053977.4BTLA, MAPT, MOG, IRF1, TNFRSF14

Publications for Subacute Sclerosing Panencephalitis

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52PubMed
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Articles related to Subacute Sclerosing Panencephalitis:

(show top 50)    (show all 497)
idTitleAuthorsYear
1
Subacute sclerosing panencephalitis: A clinical appraisal. (24339595)
2013
2
Atypical subacute sclerosing panencephalitis with short onset latency. (24222287)
2013
3
Subacute sclerosing panencephalitis masquerading as toxoplasmosis chorioretinitis. (22560432)
2012
4
Rapidly progressive subacute sclerosing panencephalitis presenting with acute loss of vision. (22368974)
2011
5
Ocular findings in subacute sclerosing panencephalitis. (21428756)
2011
6
Subacute sclerosing panencephalitis in immunized Thai children. (22619930)
2011
7
Prognosis in subacute sclerosing panencephalitis. (21745204)
2011
8
Epidemiological findings and clinical and magnetic resonance presentations in subacute sclerosing panencephalitis. (21672364)
2011
9
The F gene of the Osaka-2 strain of measles virus derived from a case of subacute sclerosing panencephalitis is a major determinant of neurovirulence. (20719945)
2010
10
Atypical presentations of subacute sclerosing panencephalitis in two neurologically handicapped cases. (20135578)
2009
11
Indication of peripheral nerve hyperexcitability in adult-onset subacute sclerosing panencephalitis (SSPE). (18483711)
2008
12
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
13
Recombinant antibodies generated from both clonal and less abundant plasma cell immunoglobulin G sequences in subacute sclerosing panencephalitis brain are directed against measles virus. (17065133)
2006
14
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
15
Long term survival in subacute sclerosing panencephalitis: an enigma. (16554134)
2006
16
Subacute sclerosing panencephalitis surveillance study in Istanbul. (16368215)
2006
17
Twenty-year follow-up of a patient with subacute sclerosing panencephalitis. (16118498)
2005
18
Laser-capture microdissection of plasma cells from subacute sclerosing panencephalitis brain reveals intrathecal disease-relevant antibodies. (15883366)
2005
19
Subacute sclerosing panencephalitis in the differential diagnosis of encephalitis. (15505172)
2004
20
Pseudotumor cerebri secondary to subacute sclerosing panencephalitis. (15605476)
2004
21
Trial of intraventricular ribavirin therapy for subacute sclerosing panencephalitis in Japan. (13129596)
2003
22
Randomized treatment study of inosiplex versus combined inosiplex and intraventricular interferon-alpha in subacute sclerosing panencephalitis (SSPE): international multicenter study. (14736075)
2003
23
Interferon therapy-responsive brain metabolic abnormalities in a case of adult-onset subacute sclerosing panencephalitis evaluated by 1H MRS analysis. (12614932)
2003
24
Clinical presentation of subacute sclerosing panencephalitis in Papua New Guinea. (12631311)
2003
25
Treatment of subacute sclerosing panencephalitis with interferon-alpha, ribavirin, and inosiplex. (12503650)
2002
26
Hypothalamic digoxin-mediated model for subacute sclerosing panencephalitis. (12161817)
2002
27
Alterations and diversity in the cytoplasmic tail of the fusion protein of subacute sclerosing panencephalitis virus strains isolated in Osaka, Japan. (12076836)
2002
28
An immunohistochemical study of the distribution of the measles virus receptors, CD46 and SLAM, in normal human tissues and subacute sclerosing panencephalitis. (11950898)
2002
29
MRI follow-up of basal ganglia involvement in subacute sclerosing panencephalitis. (11516619)
2001
30
Intraventricular alpha-interferon in subacute sclerosing panencephalitis. (9443512)
1998
31
Intrathecal production of measles-specific IgA in subacute sclerosing panencephalitis. (9272185)
1997
32
Fast-wave periodic complexes in a mentally retarded child who later developed subacute sclerosing panencephalitis: a modification of a classic EEG by preexisting brain damage? (9393892)
1997
33
Alpha-interferon in the treatment of subacute sclerosing panencephalitis. (9430312)
1997
34
MRI findings in subacute sclerosing panencephalitis. (8909443)
1996
35
Subacute sclerosing panencephalitis (SSPE): early diagnosis, prognostic factors and natural history. (8856657)
1996
36
Rapid progressive subacute sclerosing panencephalitis after perinatally acquired measles virus infection. (7715374)
1995
37
Complete nucleotide sequence of the phosphoprotein of the Yamagata-1 strain of a defective subacute sclerosing panencephalitis (SSPE) virus. (1536889)
1992
38
Subacute sclerosing panencephalitis in the developing world. (1509330)
1992
39
Chorioretinitis as a possible very early manifestation of subacute sclerosing panencephalitis. (1953517)
1991
40
The distribution of Alzheimer's neurofibrillary tangles and gliosis in chronic subacute sclerosing panencephalitis. (1698006)
1990
41
Positron emission tomography demonstrated localized luxury perfusion in subacute sclerosing panencephalitis. (2246484)
1990
42
Generalized and localized biased hypermutation affecting the matrix gene of a measles virus strain that causes subacute sclerosing panencephalitis. (2585612)
1989
43
Subacute sclerosing panencephalitis in South Africa. (2603186)
1989
44
Extended repertoire of specific antibodies in CSF of patients with subacute sclerosing panencephalitis compared to those with multiple sclerosis: anti-bacterial antibodies are also increased. (2925841)
1989
45
Multiple viral mutations rather than host factors cause defective measles virus gene expression in a subacute sclerosing panencephalitis cell line. (3346948)
1988
46
Impaired measles-specific cytotoxic T-cell response in subacute sclerosing panencephalitis. (3264687)
1988
47
HLA and C4 in subacute sclerosing panencephalitis. (3672494)
1987
48
A study of subacute sclerosing panencephalitis in general practice. (3693931)
1987
49
Subacute sclerosing panencephalitis: detection of measles antibody in serum and cerebrospinal fluid by enzyme-linked immunosorbent assay, complement fixation and hemagglutination inhibition. (3574144)
1987
50
Subacute sclerosing panencephalitis in Northern Ireland: twenty years' experience. (3811010)
1986

Variations for Subacute Sclerosing Panencephalitis

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Expression for genes affiliated with Subacute Sclerosing Panencephalitis

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2BioGPS, 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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50PathCards, 60Thomson Reuters, 55Reactome, 30KEGG, 53QIAGEN
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Pathways related to Subacute Sclerosing Panencephalitis according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.7MX1, IRF1
2
Show member pathways
9.2CD46, MX1, TNFRSF14
3
Show member pathways
9.1BTLA, TNFRSF14

Compounds for genes affiliated with Subacute Sclerosing Panencephalitis

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45Novoseek, 24HMDB, 11DrugBank
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Compounds related to Subacute Sclerosing Panencephalitis according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
12,5-oligoadenylate459.8IRF1, MX1
2poly(i-c)459.8MX1, IRF1
3ifn-alpha459.6IRF1, MX1
4silver459.5MAPT, MOG
5rantes459.5CD46, MX1, IRF1
6guanosine45 24 1111.4MX1, IRF1
7proline459.1CD46, MAPT, MOG
8cycloheximide459.0CD46, MX1, MAPT, IRF1
9serine458.5IRF1, MOG, MAPT, MX1, CD46

GO Terms for genes affiliated with Subacute Sclerosing Panencephalitis

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16Gene Ontology
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Cellular components related to Subacute Sclerosing Panencephalitis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1plasma membraneGO:0058867.8CD46, BTLA, MAPT, MOG, TNFRSF14

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

idNameGO IDScoreTop Affiliating Genes
1type I interferon signaling pathwayGO:0603379.6MX1, IRF1
2apoptotic processGO:0069159.2MX1, MAPT, IRF1
3negative regulation of alpha-beta T cell proliferationGO:0466429.1BTLA, TNFRSF14
4T cell costimulationGO:0312958.8BTLA, TNFRSF14

Molecular functions related to Subacute Sclerosing Panencephalitis according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1receptor activityGO:0048728.4CD46, BTLA, IGSF1
2protein bindingGO:0055157.7CD46, MX1, MAPT, IRF1, TNFRSF14, IGSF1

Products for genes affiliated with Subacute Sclerosing Panencephalitis

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Sources for Subacute Sclerosing Panencephalitis

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet