MCID: SBC001
MIFTS: 51

Subacute Sclerosing Panencephalitis malady

Categories: Rare diseases, Infectious diseases, Immune diseases, Neuronal diseases

Aliases & Classifications for Subacute Sclerosing Panencephalitis

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Aliases & Descriptions for Subacute Sclerosing Panencephalitis:

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

Characteristics:



Classifications:



External Ids:

Disease Ontology10 DOID:8970
ICD1027 A81.1
ICD9CM29 046.2
MeSH36 D013344
NCIt42 C85171
UMLS65 C0038522

Summaries for Subacute Sclerosing Panencephalitis

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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 dawson disease, is related to limbic encephalitis with ncmags antibodies and baylisascariasis, and has symptoms including encephalitis An important gene associated with Subacute Sclerosing Panencephalitis is CD46 (CD46 Molecule), and among its related pathways are Immune response_IL-12 signaling pathway and IL12 signaling mediated by STAT4. 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).

NIH Rare Diseases:45 Subacute sclerosing panencephalitis (sspe) a rare condition that is caused by a measles infection acquired earlier in life. signs and symptoms of the condition primarily affect the central nervous system and often develop approximately 7 to 10 years after a person recovers from the measles. affected people may initially experience behavioral changes, dementia, and disturbances in motor function. in the late stages of the disease, affected people often progress to a comatose state, and then to a persistent vegetative state. ultimately, many people with sspe succumb to fever, heart failure, or the brain's inability to continue controlling the autonomic nervous system. it is unclear why some people develop sspe after they have seemingly recovered from the measles while others do not. researchers suspect that sspe may be due to an abnormal immune response or a mutant form of the measles virus that causes a persistent infection within the central nervous system. treatment is supportive and primarily based on the signs and symptoms present in each person. recent studies have shown that certain medications (called antiviral and immunomodulatory drugs) may slow the progression of the condition, although the best treatment regimen and their long-term effects in people with sspe are currently unknown. last updated: 4/20/2016

Wikipedia:68 Subacute sclerosing panencephalitis (SSPE) also known as Dawson Disease, Dawson encephalitis, and... more...

Related Diseases for Subacute Sclerosing Panencephalitis

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

(show all 37)
idRelated DiseaseScoreTop Affiliating Genes
1limbic encephalitis with ncmags antibodies10.3IL2, IL4
2baylisascariasis10.3IL2, IL4
3epididymis cancer10.3IL2, IL4
4cd45 deficiency10.3IL2, IL4
5maxillary neoplasm10.2IL2, IL4
6collagenous colitis10.2IL2, IL4
7variola major10.2CD46, IL2
8spleen angiosarcoma10.2IL2, IL4
9b cell deficiency10.2IL2, IL4
10vulvovaginitis10.2IL2, IL4
11chronic conjunctivitis10.2IL2, IL4
12acute leukemia of ambiguous lineage10.2IL2, IL4
13human spumaretrovirus infection10.1IL2, PDCD1
14periodic limb movement disorder10.1IL2, IL4, MX1
15orbital plasma cell granuloma10.1CD46, IL2, IL4
16miliary tuberculosis10.1IL2, MX1
17nasopharyngitis10.1IFNA2, IL4
18reticulosarcoma10.1IFNA2, IL2
19tethered spinal cord syndrome10.1IL12RB1, IL2, IL4
20leech infestation10.0IL2, IL4
21hyperimmunoglobulin syndrome10.0IL12RB1, IL2, IL4
22reflex sympathetic dystrophy10.0IL4, PDCD1
23spinal polio10.0IL2, TLR3
24hyperthyroxinemia10.0IFNA2, IL2
25chronic eosinophilic pneumonia10.0IFNA2, IL2, IL4
26hodgkin's lymphoma, lymphocytic-histiocytic predominance10.0IFNA2, IL2, IL4
27exophthalmos9.9IFNA2, TLR3
28pustulosis palmaris et plantaris9.9IL4, TLR3
29opioid abuse9.9IL4, TLR3
30leukoplakia of penis9.9IL2, IL4, IRF1
31female breast lower-outer quadrant cancer9.9IFNA2, TLR3
32multiple sclerosis, disease progression, modifier of9.9IL2, IL4, MX1, PDCD1
33bestrophinopathy9.7IFNA2, IL12RB1, IL2, IL4
34ornithosis9.7MX1, TLR3
35hodgkin's lymphoma, lymphocytic depletion9.6CD46, IL2, SLAMF1, TLR3
36urethral diverticulum9.3IFNA2, IL2, IL4, MX1, TLR3
37polycystic kidney disease7.7BTLA, CD46, IFNA2, IL12RB1, IL2, IL4

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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HPO human phenotypes related to Subacute Sclerosing Panencephalitis:

id Description Frequency HPO Source Accession
1 encephalitis HP:0002383

Drugs & Therapeutics for Subacute Sclerosing Panencephalitis

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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

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Anatomical Context for Subacute Sclerosing Panencephalitis

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MalaCards organs/tissues related to Subacute Sclerosing Panencephalitis:

33
Brain, Heart, Skin, Prostate, Liver, Breast, Endothelial

FMA organs/tissues related to Subacute Sclerosing Panencephalitis:

16
Brain

Animal Models for Subacute Sclerosing Panencephalitis or affiliated genes

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MGI Mouse Phenotypes related to Subacute Sclerosing Panencephalitis:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053976.9BTLA, IL12RB1, IL2, IL4, IRF1, PDCD1
2MP:00053876.6BTLA, IL12RB1, IL2, IL4, IRF1, PDCD1

Publications for Subacute Sclerosing Panencephalitis

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Articles related to Subacute Sclerosing Panencephalitis:

(show top 50)    (show all 528)
idTitleAuthorsYear
1
Development of a one-step duplex RT-PCR method for the simultaneous detection of VP3/VP1 and VP1/P2B regions of the hepatitis A virus. (27197668)
2016
2
Keratoconjunctivitis sicca exacerbation in a dog treated with systemic atenolol. (27071733)
2016
3
Cannabidiol increases survival and promotes rescue of cognitive function in a murine model of cerebral malaria. (25595981)
2015
4
Identification of mutations, genotype-phenotype correlation and prenatal diagnosis of maple syrup urine disease in Indian patients. (26257134)
2015
5
Enzyme replacement for craniofacial skeletal defects and craniosynostosis in murine hypophosphatasia. (25959417)
2015
6
Hepatoprotective Effect of Ulinastatin in a Rat Model of Major Hepatectomy After Obstructive Jaundice. (25647759)
2015
7
Isolated double-orifice mitral valve: a case report. (26681334)
2015
8
The Relationship Between Development of Neuronal and Astrocytic Tau Pathologies in Subcortical Nuclei and Progression of Argyrophilic Grain Disease. (26439704)
2015
9
The reliability of rehabilitative ultrasound imaging in the measurement of infraspinatus muscle function in the symptomatic and asymptomatic shoulders of patients with unilateral shoulder impingement syndrome. (25883861)
2015
10
Blastomycosis presenting as a nonhealing wound. (22713783)
2012
11
Fever-like temperature modification differentially affects in vitro signaling of bradykinin B(1) and B(2) receptors. (21871009)
2011
12
Outbreak of botulism (Clostridium botulinum type C) in wild waterfowl: Seoul, Korea. (20688704)
2010
13
Four novel RSK2 mutations in females with Coffin-Lowry syndrome. (20637903)
2010
14
Reduced membrane lipids in the cortex of Alzheimer's disease transgenic mice. (18373196)
2009
15
An unusual case of malignant thymoma associated graft-versus-host disease. (21853007)
2009
16
Prostate stem cell antigen mRNA expression in preoperatively negative biopsy specimens predicts subsequent cancer after transurethral resection of the prostate for benign prostatic hyperplasia. (19462463)
2009
17
Expression and potential function of the CXC chemokine CXCL16 in pancreatic ductal adenocarcinoma. (18636150)
2008
18
Constitutive activity of JNK2 alpha2 is dependent on a unique mechanism of MAPK activation. (18940813)
2008
19
Role of wild-type estrogen receptor-beta in mitochondrial cytoprotection of cultured normal male and female human lens epithelial cells. (18577698)
2008
20
Most patients with minimal histological residuals of gastric MALT lymphoma after successful eradication of Helicobacter pylori can be managed safely by a watch and wait strategy: experience from a large international series. (17639089)
2007
21
Gene expression patterns in hepatic tissue and visceral adipose tissue of patients with non-alcoholic fatty liver disease. (17953248)
2007
22
A critical appraisal of molecular xenomonitoring as a tool for assessing progress toward elimination of Lymphatic Filariasis. (17978055)
2007
23
Cell biology and pathophysiology of the diacylglycerol kinase family: morphological aspects in tissues and organs. (17964921)
2007
24
Calcium dependent and independent cytokine synthesis by air pollution particle-exposed human bronchial epithelial cells. (17720209)
2007
25
PP2A is required for centromeric localization of Sgo1 and proper chromosome segregation. (16580887)
2006
26
Nitric oxide reduces adenosine transporter ENT1 gene (SLC29A1) promoter activity in human fetal endothelium from gestational diabetes. (16688763)
2006
27
Does H. Pylori infection play a role in idiopathic thrombocytopenic purpura and in other autoimmune diseases? (15929756)
2005
28
Molecular characterization of the commonly used human androgen receptor expression vector, pSG5-AR. (14968432)
2004
29
Myelin-, reactive glia-, and scar-derived CNS axon growth inhibitors: expression, receptor signaling, and correlation with axon regeneration. (15048847)
2004
30
Sudden infant death syndrome is not associated with the mutation of PHOX2B gene, a major causative gene of congenital central hypoventilation syndrome. (15185974)
2004
31
Morphological changes in late onset acid Maltase deficient patients with splicing gene mutation. (15088498)
2003
32
The effects of aflatoxin B1 on the development of kwashiorkor in mice. (12723897)
2003
33
Losartan in diabetic nephropathy. (12558462)
2003
34
Antimicrobial resistance of Staphylococcus aureus isolated from impetigo patients between 1994 and 2000. (12581136)
2003
35
Class VI myosin moves processively along actin filaments backward with large steps. (11779171)
2002
36
Involvement of a ferroprotein sensor in hypoxia-mediated inhibition of neutrophil apoptosis. (12351415)
2002
37
Nutrition interventions in aging and age-associated disease. (11795514)
2001
38
Rhythmic cortical and muscle discharges induced by fatigue in corticobasal degeneration. (10699413)
2000
39
Evaluation of intravenous parecoxib for the relief of acute post-surgical pain. (11060833)
2000
40
Mixed hepatoblastoma diagnosed by fine-needle aspiration biopsy cytology: a case report. (9784999)
1998
41
Iritis and hypotony after treatment with intravenous cidofovir for cytomegalovirus retinitis. (9194724)
1997
42
Cytotoxic amyloid peptides inhibit cellular 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide (MTT) reduction by enhancing MTT formazan exocytosis. (9375659)
1997
43
Lack of TGF-alpha and TGF-beta 1 synthesis by human eosinophils in chronic oral ulcers. (8784899)
1996
44
An unusual case of transient papular mucinosis associated with carpal tunnel syndrome. (8369216)
1993
45
Effect of interferon-gamma on hepatitis B viral antigen expression in primary hepatocyte culture. (1959885)
1991
46
Sequential bacteriological findings in the cerebrospinal fluid of Nigerian patients with pneumococcal meningitis. (3958504)
1986
47
Assignment of the human tyrosine aminotransferase gene to chromosome 16. (2870018)
1986
48
Branhamella catarrhalis (beta lactamase positive) ophthalmia neonatorum. (6147687)
1984
49
Skin tests in a primate model of allergic bronchopulmonary aspergillosis. (7016762)
1981
50
INTERMITTENT ANOMALOUS ATRIOVENTRICULAR EXCITATION. (14265381)
1965

Variations for Subacute Sclerosing Panencephalitis

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Expression for genes affiliated with Subacute Sclerosing Panencephalitis

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

Pathways for genes affiliated with Subacute Sclerosing Panencephalitis

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Pathways related to Subacute Sclerosing Panencephalitis according to GeneCards Suite gene sharing:

(show all 23)
idSuper pathwaysScoreTop Affiliating Genes
1
Show member pathways
9.8IL12RB1, IRF1
29.5IL2, IRF1
39.4IL2, IL4
49.4IL2, IL4
59.4IL2, IL4
69.4IL2, IL4
7
Show member pathways
9.4IFNA2, MX1
8
Show member pathways
9.2IL12RB1, IL4, TLR3
99.2BTLA, PDCD1, SLAMF1
109.1IL2, IL4, IRF1
11
Show member pathways
9.1IL12RB1, IL2, IL4
129.1IL12RB1, IL2, IL4
13
Show member pathways
9.0IL12RB1, IL2, TLR3
148.8IFNA2, IRF1, TLR3
158.7BTLA, IL4, PDCD1, SLAMF1
168.7IFNA2, IL2, SLAMF1
178.7IFNA2, IL12RB1, IL2
188.3IFNA2, IL2, IL4, SLAMF1
19
Show member pathways
8.2IFNA2, IL12RB1, IL2, IL4
20
Show member pathways
8.2IFNA2, IL12RB1, IL2, IL4
21
Show member pathways
8.0IFNA2, IL2, IL4, IRF1, MX1
22
Show member pathways
7.2CD46, IFNA2, IL2, IL4, MX1, SLAMF1
23
Show member pathways
6.3BTLA, CD46, IFNA2, IL12RB1, IL2, IRF1

GO Terms for genes affiliated with Subacute Sclerosing Panencephalitis

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

(show all 20)
idNameGO IDScoreTop Affiliating Genes
1positive regulation of interferon-beta productionGO:003272810.3IRF1, TLR3
2positive regulation of interleukin-10 productionGO:003273310.3CD46, IL4
3positive regulation of chemokine biosynthetic processGO:004508010.2IL4, TLR3
4positive regulation of T cell differentiationGO:004558210.1IL2, IL4
5extrinsic apoptotic signaling pathway in absence of ligandGO:009719210.0IL2, IL4
6positive regulation of tyrosine phosphorylation of Stat5 proteinGO:004252310.0IL2, IL4
7positive regulation of B cell proliferationGO:003089010.0IL2, IL4
8negative regulation of osteoclast differentiationGO:004567110.0IL4, TLR3
9positive regulation of T cell proliferationGO:004210210.0IL2, IL4
10microglial cell activationGO:00017749.9IL4, TLR3
11positive regulation of interferon-gamma productionGO:00327299.9IL12RB1, IL2
12positive regulation of isotype switching to IgG isotypesGO:00483049.9IL2, IL4
13cellular response to interferon-gammaGO:00713469.9IL12RB1, TLR3
14B cell differentiationGO:00301839.8IFNA2, IL4
15response to exogenous dsRNAGO:00433309.7IFNA2, TLR3
16positive regulation of protein phosphorylationGO:00019349.2IL2, IL4, TLR3
17defense responseGO:00069529.2IFNA2, MX1, TLR3
18positive regulation of cell proliferationGO:00082849.0IL2, IL4, SLAMF1
19innate immune responseGO:00450878.4CD46, IL2, MX1, SLAMF1, TLR3
20defense response to virusGO:00516078.2IFNA2, IRF1, MX1, TLR3

Sources for Subacute Sclerosing Panencephalitis

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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