SSPE
MCID: SBC001
MIFTS: 50

Subacute Sclerosing Panencephalitis (SSPE) malady

Infectious, Immune categories

Summaries for Subacute Sclerosing Panencephalitis

Sources:
8Disease Ontology, 44NINDS, 64Wikipedia, 47OMIM, 33MalaCards
See all sources

Fully expand this MalaCard

Export this MalaCard
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 mumps. 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 cortex, 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:64 Subacute sclerosing panencephalitis (SSPE) is a rare chronic, progressive encephalitis that affects... more...

Description from OMIM:47 260470

Aliases & Classifications for Subacute Sclerosing Panencephalitis

Sources:
8Disease Ontology, 43NIH Rare Diseases, 44NINDS, 10DISEASES, 45Novoseek, 61UMLS, 27ICD9CM, 57SNOMED-CT, 35MeSH, 47OMIM, 40NCIt, 25ICD10
See all sources

Classifications:

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


Aliases & Descriptions:

subacute sclerosing panencephalitis 8 43 44 10 45 61
van bogaert's sclerosing leukoencephalitis 8
subacute sclerosing leukoencephalopathy 8
immunosuppressive measles encephalitis 8
sspe 43


External Ids:

Disease Ontology8 DOID:8970
ICD9CM27 046.2
MeSH35 D013344
OMIM47 260470
NCIt40 C85171
ICD1025 A81.1

Related Diseases for Subacute Sclerosing Panencephalitis

Sources:
17GeneCards, 18GeneDecks
See all sources

Diseases related to Subacute Sclerosing Panencephalitis via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 91)
idRelated DiseaseScoreTop Affiliating Genes
1measles31.9TLR3, GNLY, IRF1, CD46, MX1, SLAMF1
2mumps30.6IRF1
3tuberculosis30.4IFNGR1, GNLY, IRF1, CXCL10, IL12RB1
4myoclonus10.7
5acute disseminated encephalomyelitis10.6
6brain disease10.6
7rubella10.5
8cortical blindness10.5
9blindness10.5
10rubella panencephalitis10.5
11status epilepticus10.4
12pseudotumor cerebri10.4
13acquired immunodeficiency syndrome10.4
14progressive multifocal leukoencephalopathy10.4
15toxoplasmosis10.4
16dawson disease10.4
17subacute sclerosing leukoencephalitis10.4
18parkinson's disease10.3
19postencephalitic parkinson disease10.3
20neuromyelitis optica10.3
21aseptic meningitis10.3
22borna disease10.3
23exophthalmos10.3
24conduct disorder10.3
25viral encephalitis10.3
26alzheimer's disease10.3
27listeriosis10.3
28adult syndrome10.3
29aphasia10.3
30stomatitis10.3
31neurologic diseases10.3
32limbic encephalitis10.3
33balint syndrome10.3
34autonomic dysfunction10.0
35lupus erythematosus10.0MX1
36myositis10.0MAPT
37cryoglobulinemic vasculitis10.0IFNA1
38severe acute respiratory syndrome10.0CXCL10
39lichen planus10.0MX1
40nephritis10.0LIF
41dermatomyositis10.0MX1
42biliary atresia10.0MX1, CXCL10
43cryoglobulinemia10.0IFNA1
44vasculitis10.0CD46
45myocarditis10.0IL12RB1, IRF1
46psoriasis10.0MX1, CXCL10, IRF1
47relapsing-remitting multiple sclerosis10.0MX1, IFNA1, MOG
48mycobacterium avium complex disease10.0IFNGR1
49newcastle disease10.0CXCL10, IRF1, IFNA1
50kaposi's sarcoma10.0IFNA1

Graphical network of the top 20 diseases related to Subacute Sclerosing Panencephalitis:



Diseases related to subacute sclerosing panencephalitis

Clinical Features for Subacute Sclerosing Panencephalitis

Sources:
47OMIM
See all sources

Clinical features from OMIM:

260470

Drugs & Therapeutics for Subacute Sclerosing Panencephalitis

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Subacute Sclerosing Panencephalitis

Drug clinical trials:

Search ClinicalTrials for Subacute Sclerosing Panencephalitis

Search NIH Clinical Center for Subacute Sclerosing Panencephalitis

Search CenterWatch for Subacute Sclerosing Panencephalitis

Genetic Tests for Subacute Sclerosing Panencephalitis

Anatomical Context for Subacute Sclerosing Panencephalitis

Sources:
14FMA, 33MalaCards
See all sources

MalaCards organs/tissues related to Subacute Sclerosing Panencephalitis:

33
Brain, Cortex, Heart, T cells, B cells, Endothelial, Temporal lobe

FMA organs/tissues related to Subacute Sclerosing Panencephalitis:

14
Brain

Animal Models for Subacute Sclerosing Panencephalitis or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
See all sources

MGI Mouse Phenotypes related to Subacute Sclerosing Panencephalitis:

37
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

Sources:
51PubMed
See all sources

Articles related to Subacute Sclerosing Panencephalitis:

(show top 50)    (show all 500)
idTitleAuthorsYear
1
Oxidant and Antioxidant Status in Children With Subacute Sclerosing Panencephalitis. (23872915)
2013
2
Subacute sclerosing panencephalitis presenting as neuromyelitis optica. (23242079)
2012
3
Survey of subacute sclerosing panencephalitis in Japan. (22408143)
2012
4
Atypical presentation of adult-age onset subacute sclerosing panencephalitis. (22187214)
2012
5
Rapidly progressive subacute sclerosing panencephalitis presenting with acute loss of vision. (22368974)
2011
6
Subacute sclerosing panencephalitis in papua new guinean children: the cost of continuing inadequate measles vaccine coverage. (21245918)
2011
7
Brainstem involvement in subacute sclerosing panencephalitis. (21483132)
2011
8
Brainstem involvement in subacute sclerosing panencephalitis. (21434543)
2010
9
Subacute sclerosing panencephalitis: an update. (20561004)
2010
10
Atypical presentations of subacute sclerosing panencephalitis in two neurologically handicapped cases. (20135578)
2009
11
Fulminant adult-onset subacute sclerosing panencephalitis: a case report. (21686559)
2009
12
Reduced gray matter volume in the frontotemporal cortex of patients with early subacute sclerosing panencephalitis. (18854439)
2009
13
Analysis of progressive ophthalmic lesion in a patient with subacute sclerosing panencephalitis. (18203105)
2008
14
Cerebrospinal fluid levels of matrix metalloproteinase-9 and tissue inhibitor of metalloproteinase-1 in subacute sclerosing panencephalitis. (18395263)
2008
15
Interleukin (IL)-12, IL-2, interferon-gamma gene polymorphisms in subacute sclerosing panencephalitis patients. (17994425)
2007
16
Subacute sclerosing panencephalitis (SSPE): an insight into the diagnostic errors from a tertiary care university hospital. (17641252)
2007
17
MR imaging, diffusion-weighted imaging and MR spectroscopy findings in acute rapidly progressive subacute sclerosing panencephalitis. (17098390)
2007
18
Atypical subacute sclerosing panencephalitis: case report. (18094871)
2007
19
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
20
Inosiplex affects the spectra of proton magnetic resonance spectroscopy in subacute sclerosing panencephalitis. (16566890)
2006
21
Quantification of neurometabolites in subacute sclerosing panencephalitis by 1H-MRS. (16966569)
2006
22
F-18 FDG and F-18 FDOPA PET brain imaging in subacute sclerosing panencephalitis. (15965337)
2005
23
Detection of interleukin 10 in cerebrospinal fluid of patients with subacute sclerosing panencephalitis. (15804961)
2005
24
Atypical EEG findings in subacute sclerosing panencephalitis. (15982925)
2005
25
Subacute sclerosing panencephalitis in the differential diagnosis of encephalitis. (15505172)
2004
26
Report of two children with subacute sclerosing panencephalitis displaying an atypical clinical course. (15028344)
2004
27
Familial subacute sclerosing panencephalitis in two siblings. (15464644)
2004
28
Subacute sclerosing panencephalitis: a preventable disease that needs to be prevented. (15505147)
2004
29
Trial of intraventricular ribavirin therapy for subacute sclerosing panencephalitis in Japan. (13129596)
2003
30
Hypothalamic digoxin-mediated model for subacute sclerosing panencephalitis. (12161817)
2002
31
Contribution of the interleukin 4 gene to susceptibility to subacute sclerosing panencephalitis. (12020266)
2002
32
Combined treatment with interferon-alpha and ribavirin for subacute sclerosing panencephalitis. (11182282)
2001
33
Subacute sclerosing panencephalitis, a measles complication, in an internationally adopted child. (10905971)
2000
34
Case report and molecular analysis of subacute sclerosing panencephalitis in a South African Child. (9986851)
1999
35
Adult-onset subacute sclerosing panencephalitis: case reports and review of the literature. (9159729)
1997
36
Leukaemia inhibitory factor mRNA is expressed in the brains of patients with subacute sclerosing panencephalitis. (9209269)
1997
37
Subacute sclerosing panencephalitis manifesting as viral retinitis: clinical and histopathologic findings. (9124250)
1997
38
Widespread, restricted low-level measles virus infection of brain in a case of subacute sclerosing panencephalitis. (8787145)
1996
39
Carbamazepine and myoclonus in SSPE subacute sclerosing panencephalitis. (8962596)
1996
40
Subacute sclerosing panencephalitis. (8969008)
1996
41
Ferritin, creatine kinase, and neopterin in subacute sclerosing panencephalitis. (1492651)
1992
42
Resolving MRI abnormalities with progression of subacute sclerosing panencephalitis. (2046910)
1991
43
Subacute sclerosing panencephalitis in England and Wales: transient effects and risk estimates. (1792467)
1991
44
Risk of subacute sclerosing panencephalitis from measles vaccination. (2263442)
1990
45
Intraventricular interferon treatment for subacute sclerosing panencephalitis. (2918207)
1989
46
Structural defect linked to nonrandom mutations in the matrix gene of biken strain subacute sclerosing panencephalitis virus defined by cDNA cloning and expression of chimeric genes. (2915379)
1989
47
Electroencephalographic changes during interferon therapy in a case of subacute sclerosing panencephalitis. (2468499)
1989
48
Intrathecal interferon in subacute sclerosing panencephalitis. (3227800)
1988
49
Magnetic resonance imaging in infections of the brain: findings in tuberculosis, listeriosis, toxoplasmosis, subacute sclerosing panencephalitis, and multiple sclerosis. (3455473)
1987
50
Restricted expression of measles virus proteins in brains from cases of subacute sclerosing panencephalitis. (3537205)
1986

Genetic Variations for Subacute Sclerosing Panencephalitis

Expression for genes affiliated with Subacute Sclerosing Panencephalitis

Sources:
1BioGPS, 15Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Subacute Sclerosing Panencephalitis

Search GEO for disease gene expression data for Subacute Sclerosing Panencephalitis.

Pathways for genes affiliated with Subacute Sclerosing Panencephalitis

Sources:
52QIAGEN, 12EMD Millipore, 54Reactome, 4Cell Signaling Technology, 38NCBI BioSystems Database, 53R&D Systems, 30KEGG
See all sources

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
Hide members
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

Sources:
45Novoseek, 50PharmGKB, 11DrugBank, 29IUPHAR, 60Tocris Bioscience, 24HMDB, 2BitterDB
See all sources

Compounds related to Subacute Sclerosing Panencephalitis according to GeneCards/GeneDecks:

(show all 24)
idCompoundScoreTop Affiliating Genes
1rebif4510.6MX1, IFNA1
2ribavirin45 50 1112.6MX1
3avonex4510.6MX1, IFNA1
4imiquimod45 29 60 1113.6TLR3, IFNA1, MX1
5betaseron4510.6MX1, IFNA1
6inosine monophosphate4510.5TLR3, IFNA1, IRF1
72,5-oligoadenylate4510.5TLR3, IFNA1, IRF1, CXCL10, MX1
8poly(i-c)4510.5TLR3, IFNA1, IRF1, CXCL10, MX1
9rantes4510.5CD46, LIF, IRF1, IFNA1, CCL16
10polyinosinic-polycytidylic acid4510.5TLR3, IFIH1, IFNA1, IRF1, CXCL10
11cycloheximide4510.5IFNA1, IRF1, MAPT, CXCL10, MX1
12dexamethasone45 50 29 1113.4TLR3, IFNA1, LIF, CD46
13ifn-alpha4510.4TLR3, IFNA1, IRF1, LIF, CXCL10, MX1
14resiquimod45 6011.4IFNA1, TLR3
15neopterin4510.4MX1, CXCL10, IFNA1
16ccl345 2911.4CCL16, IFNA1, CXCL10
17nitric oxide45 11 2412.3MOG, TLR3, IRF1, LIF, CD46, CXCL10
18retinoic acid45 2411.3TLR3, IFIH1, IFNA1, IRF1, LIF, MAPT
19serine4510.3MOG, TLR3, GNLY, IRF1, LIF, MAPT
20chloroquine45 2 50 29 1114.3IFNA1, MAPT, CXCL10
21actinomycin d4510.2MAPT, LIF, IRF1, IFNA1
22tyrosine4510.2TLR3, IFNGR1, IFNA1, IRF1, LIF, CD46
23gp 1304510.2IL12RB1, LIF, IFNGR1
24glatiramer acetate45 1111.0MOG, IFNA1

GO Terms for genes affiliated with Subacute Sclerosing Panencephalitis

Sources:
16Gene Ontology
See all sources

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

  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Subacute Sclerosing Panencephalitis

3CDC
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet