MCID: SBC001
MIFTS: 43

Subacute Sclerosing Panencephalitis malady

Rare diseases, Infectious diseases, Immune diseases, Neuronal diseases categories

Aliases & Classifications for Subacute Sclerosing Panencephalitis

About this section

Aliases & Descriptions for Subacute Sclerosing Panencephalitis:

Name: Subacute Sclerosing Panencephalitis 10 45 46 47 12 65 36
Van Bogaert's Sclerosing Leukoencephalitis 10
Subacute Sclerosing Leukoencephalopathy 10
 
Immunosuppressive Measles Encephalitis 10
Sspe 45


Classifications:



External Ids:

Disease Ontology10 DOID:8970
NCIt42 C85171
MeSH36 D013344
ICD9CM29 046.2
ICD1027 A81.1

Summaries for Subacute Sclerosing Panencephalitis

About this section
NINDS:46 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 based summary: Subacute Sclerosing Panencephalitis, also known as van bogaert's sclerosing leukoencephalitis, is related to acquired immunodeficiency syndrome and measles, and has symptoms including autosomal recessive inheritanceand encephalitis. An important gene associated with Subacute Sclerosing Panencephalitis is CD46 (CD46 Molecule, Complement Regulatory Protein), and among its related pathways are Immune response_IL-12 signaling pathway and Peginterferon alpha-2a/Peginterferon alpha-2b Pathway (Hepatocyte), Pharmacodynamics. Affiliated tissues include brain, brain and heart, and related mouse phenotypes are hematopoietic system and immune system.

Disease Ontology:10 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).

Related Diseases for Subacute Sclerosing Panencephalitis

About this section

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



Diseases related to subacute sclerosing panencephalitis

Symptoms for Subacute Sclerosing Panencephalitis

About this section

HPO human phenotypes related to Subacute Sclerosing Panencephalitis:

id Description Frequency HPO Source Accession
1 autosomal recessive inheritance HP:0000007
2 encephalitis HP:0002383

Drugs & Therapeutics for Subacute Sclerosing Panencephalitis

About this section

Interventional clinical trials:

idNameStatusNCT IDPhase
1Evaluating Dietary Intervention Before surgicaL Treatment for EpilepsyNot yet recruitingNCT02261753Phase 4
2Prevention of Serious Adverse Events in Acute Care Hospitals by Afferent Limb and Response Method Intervention - the ALARM Intervention Study.Active, not recruitingNCT01949025

Search NIH Clinical Center for Subacute Sclerosing Panencephalitis


Cochrane evidence based reviews: Subacute Sclerosing Panencephalitis

Genetic Tests for Subacute Sclerosing Panencephalitis

About this section

Anatomical Context for Subacute Sclerosing Panencephalitis

About this section

MalaCards organs/tissues related to Subacute Sclerosing Panencephalitis:

33
Brain, Heart, T cells, Cortex, Endothelial

FMA organs/tissues related to Subacute Sclerosing Panencephalitis:

16
Brain

Animal Models for Subacute Sclerosing Panencephalitis or affiliated genes

About this section

MGI Mouse Phenotypes related to Subacute Sclerosing Panencephalitis:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053976.9IL12RB1, IL4, IRF1, MBP, MOG, PDCD1
2MP:00053876.6IL12RB1, IL4, IRF1, MBP, MOG, PDCD1

Publications for Subacute Sclerosing Panencephalitis

About this section

Articles related to Subacute Sclerosing Panencephalitis:

(show top 50)    (show all 525)
idTitleAuthorsYear
1
The phosphoprotein genes of measles viruses from subacute sclerosing panencephalitis cases encode functional as well as non-functional proteins and display reduced editing. (26428304)
2015
2
A Decrease of Regulatory T Cells and Altered Expression of NK Receptors Are Observed in Subacute Sclerosing Panencephalitis. (25379970)
2014
3
Neuronal loss is an early component of subacute sclerosing panencephalitis. (25085642)
2014
4
Subacute sclerosing panencephalitis: A clinical appraisal. (24339595)
2013
5
Atypical subacute sclerosing panencephalitis with short onset latency. (24222287)
2013
6
Subacute sclerosing panencephalitis masquerading as toxoplasmosis chorioretinitis. (22560432)
2012
7
Rapidly progressive subacute sclerosing panencephalitis presenting with acute loss of vision. (22368974)
2011
8
Ocular findings in subacute sclerosing panencephalitis. (21428756)
2011
9
Subacute sclerosing panencephalitis in immunized Thai children. (22619930)
2011
10
Prognosis in subacute sclerosing panencephalitis. (21745204)
2011
11
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
12
Atypical presentations of subacute sclerosing panencephalitis in two neurologically handicapped cases. (20135578)
2009
13
Indication of peripheral nerve hyperexcitability in adult-onset subacute sclerosing panencephalitis (SSPE). (18483711)
2008
14
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
15
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
16
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
17
Long term survival in subacute sclerosing panencephalitis: an enigma. (16554134)
2006
18
Twenty-year follow-up of a patient with subacute sclerosing panencephalitis. (16118498)
2005
19
Laser-capture microdissection of plasma cells from subacute sclerosing panencephalitis brain reveals intrathecal disease-relevant antibodies. (15883366)
2005
20
Subacute sclerosing panencephalitis in the differential diagnosis of encephalitis. (15505172)
2004
21
Pseudotumor cerebri secondary to subacute sclerosing panencephalitis. (15605476)
2004
22
Trial of intraventricular ribavirin therapy for subacute sclerosing panencephalitis in Japan. (13129596)
2003
23
Randomized treatment study of inosiplex versus combined inosiplex and intraventricular interferon-alpha in subacute sclerosing panencephalitis (SSPE): international multicenter study. (14736075)
2003
24
Interferon therapy-responsive brain metabolic abnormalities in a case of adult-onset subacute sclerosing panencephalitis evaluated by 1H MRS analysis. (12614932)
2003
25
Clinical presentation of subacute sclerosing panencephalitis in Papua New Guinea. (12631311)
2003
26
Treatment of subacute sclerosing panencephalitis with interferon-alpha, ribavirin, and inosiplex. (12503650)
2002
27
Hypothalamic digoxin-mediated model for subacute sclerosing panencephalitis. (12161817)
2002
28
Alterations and diversity in the cytoplasmic tail of the fusion protein of subacute sclerosing panencephalitis virus strains isolated in Osaka, Japan. (12076836)
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

About this section

Expression for genes affiliated with Subacute Sclerosing Panencephalitis

About this section
Search GEO for disease gene expression data for Subacute Sclerosing Panencephalitis.

Pathways for genes affiliated with Subacute Sclerosing Panencephalitis

About this section

GO Terms for genes affiliated with Subacute Sclerosing Panencephalitis

About this section

Cellular components related to Subacute Sclerosing Panencephalitis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cell surfaceGO:00099869.0CD46, MOG, SLAMF1, TLR3
2external side of plasma membraneGO:00098978.7IL12RB1, IL4, PDCD1, SLAMF1

Biological processes related to Subacute Sclerosing Panencephalitis according to GeneCards Suite gene sharing:

(show all 14)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of memory T cell differentiationGO:004338210.3CD46, IL12RB1
2positive regulation of interferon-beta productionGO:003272810.2IRF1, TLR3
3positive regulation of interleukin-10 productionGO:003273310.2CD46, IL4
4positive regulation of chemokine biosynthetic processGO:004508010.1IL4, TLR3
5positive regulation of activated T cell proliferationGO:004210410.0IL12RB1, IL4
6negative regulation of osteoclast differentiationGO:00456719.9IL4, TLR3
7viral entry into host cellGO:00467189.9CD46, MOG, SLAMF1
8type I interferon signaling pathwayGO:00603379.6IFNA2, IRF1, MX1
9response to exogenous dsRNAGO:00433309.4IFNA2, TLR3
10defense responseGO:00069529.2IFNA2, MX1, TLR3
11defense response to virusGO:00516078.7IFNA2, IRF1, MX1, TLR3
12cytokine-mediated signaling pathwayGO:00192218.7IFNA2, IL12RB1, IRF1, MX1
13signal transductionGO:00071658.2IL12RB1, MX1, PDCD1, SLAMF1, TLR3
14apoptotic processGO:00069157.8IFNA2, IRF1, MX1, PDCD1, TLR3

Molecular functions related to Subacute Sclerosing Panencephalitis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1cytokine receptor bindingGO:00051269.6IFNA2, IL4
2receptor activityGO:00048729.3CD46, SLAMF1, TLR3
3virus receptor activityGO:00016189.2CD46, MOG, SLAMF1

Sources for Subacute Sclerosing Panencephalitis

About this section
2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet