PML
MCID: PRG009
MIFTS: 57

Progressive Multifocal Leukoencephalopathy (PML) malady

Infectious diseases, Neuronal diseases categories

Summaries for Progressive Multifocal Leukoencephalopathy

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8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 63Wikipedia, 32MalaCards
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NIH Rare Diseases:42 Progressive multifocal leukoencephalopathy (pml) is a neurological disorder that damages the myelin that covers and protects nerves in the white matter of the brain. it is caused by the jc virus (jcv). by age 10, most people have been infected with this virus, but it rarely causes symptoms unless the immune system becomes severely weakened. the disease occurs, rarely, in organ transplant patients; people undergoing chronic corticosteroid or immunosuppressive therapy; and individuals with cancer, such as hodgkin’s disease, lymphoma, and sarcoidosis.  pml is most common among individuals with acquired immune deficiency syndrome (aids). last updated: 4/1/2011

MalaCards: Progressive Multifocal Leukoencephalopathy, also known as leukoencephalopathy, progressive multifocal, is related to multiple sclerosis and leukemia. An important gene associated with Progressive Multifocal Leukoencephalopathy is IGHMBP2 (immunoglobulin mu binding protein 2), and among its related pathways is purine nucleotides degradation II (aerobic). The compounds deoxyinosine and 9-(beta-d-arabinofuranosyl)guanine have been mentioned in the context of this disorder. Affiliated tissues include brain, t cells and kidney, and related mouse phenotypes are skeleton and respiratory system.

Disease Ontology:8 A viral infectious disease that involves reactivation of jc polyomavirus in immune-compromised individuals which causes the loss of white matter (which is made up of myelin, a substance the surrounds and protects nerve fibers) in multiple areas of the brain. the symptoms include hemiparesis, aphasia, dysarthria, hemianopia, cognitive impairment and coma.

NINDS:43 Progressive multifocal leukoencephalopathy (PML) is a disease of the white matter of the brain, caused by a virus infection that targets cells that make myelin--the material that insulates nerve cells (neurons).

Wikipedia:63 Progressive multifocal leukoencephalopathy (PML), also known as progressive multifocal... more...

Aliases & Classifications for Progressive Multifocal Leukoencephalopathy

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8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 10DISEASES, 48Orphanet, 60UMLS, 44Novoseek, 34MeSH, 27ICD9CM, 39NCIt, 56SNOMED-CT, 61UMLS via Orphanet, 35MESH via Orphanet, 57SNOMED-CT via Orphanet, 25ICD10
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Classifications:

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


Characteristics (Orphanet epidemiological data):

48
progressive multifocal leukoencephalopathy:
Inheritance: Sporadic; Age of onset: Variable


Aliases & Descriptions:

progressive multifocal leukoencephalopathy 8 42 43 10 48
leukoencephalopathy, progressive multifocal 42 60
leukoencephalopathy progressive multifocal 44
progressive multifocal leukoencephalitis 48
pml 42


External Ids:

Disease Ontology8 DOID:643
MeSH34 D007968
ICD9CM27 046.3
NCIt39 C26815
SNOMED-CT56 22255007
UMLS via Orphanet61 C0023524
MESH via Orphanet35 D007968
SNOMED-CT via Orphanet57 22255007
ICD1025 A81.2

Related Diseases for Progressive Multifocal Leukoencephalopathy

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17GeneCards, 18GeneDecks
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Diseases related to Progressive Multifocal Leukoencephalopathy via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 175)
idRelated DiseaseScoreTop Affiliating Genes
1multiple sclerosis31.2PDCD1, ITGA4, LBR
2leukemia31.0PNP, ITGA4, CD9, SPIB, LBR, MECP2
3systemic lupus erythematosus30.9LBR, ADA, PDCD1, ITGA4
4acquired immunodeficiency syndrome30.8CARTPT, TAT
5arthritis30.7TAT, ITGA4
6rheumatoid arthritis30.7ADA, PDCD1, PNP, LBR, ITGA4
7myeloma30.6CD9, ITGA4
8non-hodgkin lymphoma30.5CT49, ITGA4, LBR, ADA, PNP, TAT
9lymphopenia30.4ADA, PNP, LBR
10kaposi's sarcoma30.2LBR, CD9, TAT
11oligodendroglioma30.2CT49
12purine nucleoside phosphorylase deficiency30.2ADA
13aseptic meningitis30.2ADA, CD9
14hepatitis c30.2CD9
15encephalitis30.2ITGA4, CD9, TAT
16acute lymphocytic leukemia30.2ADA, PNP, CD9, ITGA4
17mycosis fungoides30.2ITGA4, ADA
18schizophrenia30.2MECP2, FEZ1, LBR, POU3F1, PNP
19primary biliary cirrhosis30.2LBR
20retinoblastoma30.2TICAM2, CT49, MECP2
21dementia29.9TAT, CD9
22lupus erythematosus10.6
23sarcoidosis10.6
24chronic lymphocytic leukemia10.5
25adult syndrome10.4
26myeloid leukemia10.4
27crohn's disease10.4
28neuronitis10.4
29hodgkin's lymphoma10.4
30acute promyelocytic leukemia10.4
31acute myeloid leukemia10.3
32prostatitis10.3
33wiskott-aldrich syndrome10.3
34common variable immunodeficiency10.3
35t lymphocyte deficiency10.3
36cortical blindness10.3
37acute leukemia10.3
38central nervous system disease10.3
39meningitis10.3
40multiple myeloma10.3
41nervous system disease10.3
42psoriasis10.3
43vasculitis10.3
44rabies10.3
45breast cancer10.3
46prostate cancer10.3
47sarcoma10.3
48parotitis10.2
49nasopharyngitis10.2
50influenza10.2

Graphical network of the top 20 diseases related to Progressive Multifocal Leukoencephalopathy:



Diseases related to progressive multifocal leukoencephalopathy

Clinical Features for Progressive Multifocal Leukoencephalopathy

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Drugs & Therapeutics for Progressive Multifocal Leukoencephalopathy

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Progressive Multifocal Leukoencephalopathy

Drug clinical trials:

Search ClinicalTrials for Progressive Multifocal Leukoencephalopathy

Search NIH Clinical Center for Progressive Multifocal Leukoencephalopathy

Search CenterWatch for Progressive Multifocal Leukoencephalopathy

Genetic Tests for Progressive Multifocal Leukoencephalopathy

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Anatomical Context for Progressive Multifocal Leukoencephalopathy

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32MalaCards
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MalaCards organs/tissues related to Progressive Multifocal Leukoencephalopathy:

32
Brain, T cells, Kidney, Lung, Liver, B cells, Bone, Bone marrow, Spinal cord, Testes, Heart, Cortex

Animal Models for Progressive Multifocal Leukoencephalopathy or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Progressive Multifocal Leukoencephalopathy:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053909.8CARTPT, PDCD1, NFIX, CD9
2MP:00053889.6PURA, LBR, MECP2, IGHMBP2, POU3F1, CD9
3MP:00053849.2PDCD1, LBR, MECP2, ITGA4, IGBP1, PNP
4MP:00053979.2LBR, PDCD1, CARTPT, PURA, ITGA4, IGBP1
5MP:00053869.2PURA, CARTPT, FEZ1, LBR, MECP2, NFIX
6MP:00053789.1PURA, CARTPT, FEZ1, LBR, MECP2, NFIX
7MP:00107689.0MECP2, LBR, PDCD1, PURA, ITGA4, NFIX
8MP:00053878.9ADA, TICAM2, PURA, CARTPT, PDCD1, LBR
9MP:00036318.9PURA, PDCD1, FEZ1, LBR, MECP2, NFIX

Publications for Progressive Multifocal Leukoencephalopathy

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50PubMed
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Articles related to Progressive Multifocal Leukoencephalopathy:

(show top 50)    (show all 517)
idTitleAuthorsYear
1
Immune reconstitution inflammatory syndrome in a patient treated with natalizumab presenting progressive multifocal leukoencephalopathy. (23228283)
2013
2
JCV-negative natalizumab-associated progressive multifocal leukoencephalopathy: a clinico-radiological diagnosis. (22920632)
2013
3
Progressive multifocal leukoencephalopathy in an immunocompetent patient? (24163670)
2013
4
Progressive multifocal leukoencephalopathy in an HIV-negative patient following treatment with rituximab. (23882904)
2013
5
Disproportionality signal of progressive multifocal leukoencephalopathy: monoclonal antibodies versus other immunosuppressants. (23554080)
2013
6
A second-generation ELISA (STRATIFY JCVa8c DxSelecta8c) for detection of JC virus antibodies in human serum and plasma to support progressive multifocal leukoencephalopathy risk stratification. (23465394)
2013
7
Natalizumab-associated reversible encephalopathy syndrome mimicking progressive multifocal leukoencephalopathy. (22596228)
2013
8
Frequent infection of neurons by SV40 virus in SIV-infected macaque monkeys with progressive multifocal leukoencephalopathy and meningoencephalitis. (24095925)
2013
9
Disease course and outcome of 15 monocentrically treated natalizumab-associated progressive multifocal leukoencephalopathy patients. (23606731)
2013
10
Incidence and risk factors for progressive multifocal leukoencephalopathy among patients with selected rheumatic diseases. (22162369)
2012
11
Hemianopia and visual loss due to progressive multifocal leukoencephalopathy in natalizumab-treated multiple sclerosis. (22888211)
2012
12
Predicting risk of progressive multifocal leukoencephalopathy from natalizumab. (22591300)
2012
13
Progressive multifocal leukoencephalopathy in liver transplant recipients: a case report and review of the literature. (21134241)
2011
14
Rituximab-associated progressive multifocal leukoencephalopathy in rheumatoid arthritis. (21555606)
2011
15
Dual therapy with cidofovir and mirtazapine for progressive multifocal leukoencephalopathy in a sarcoidosis patient. (22114583)
2011
16
Treatment of progressive multifocal leukoencephalopathy and idiopathic CD4+ lymphocytopenia. (20961908)
2010
17
Comment to: Progressive Multifocal Leukoencephalopathy and Other Disorders Caused by JC Virus: Clinical Features and Pathogenesis : C. D. Tan, I. J. Koralnik Lancet Neurol 2010;9:425-37. (20512302)
2010
18
Progressive multifocal leukoencephalopathy in individuals with minimal or occult immunosuppression. (19828476)
2010
19
Use of diffusion-weighted imaging to evaluate the initial response of progressive multifocal leukoencephalopathy to highly active antiretroviral therapy: early experience. (20360338)
2010
20
Detection of WU polyomavirus in cerebrospinal fluid specimen from a patient with AIDS and suspected progressive multifocal leukoencephalopathy. (19530941)
2009
21
Lymphotropic polyomavirus and progressive multifocal leukoencephalopathy. (19122182)
2009
22
Polyomaviruses other than JCV are not detected in progressive multifocal leukoencephalopathy. (19447072)
2009
23
Improved detection of JC virus in AIDS patients with progressive multifocal leukoencephalopathy by T-antigen specific fluorescence resonance energy transfer hybridization probe real-time PCR: evidence of diverse JC virus genotypes associated with progressive multifocal leukoencephalopathy in Southern Africa. (19774679)
2009
24
Progressive multifocal leukoencephalopathy after natalizumab monotherapy. (19741229)
2009
25
Progressive multifocal leukoencephalopathy in a patient with Franklin disease and hypogammaglobulinemia. (19428026)
2009
26
Progressive multifocal leukoencephalopathy, efalizumab, and immunosuppression: a cautionary tale for dermatologists. (19687432)
2009
27
Diffusion-weighted imaging in patients with progressive multifocal leukoencephalopathy. (18324406)
2008
28
Progressive multifocal leukoencephalopathy - the importance of early diagnosis illustrated in four cases. (18852230)
2008
29
Progressive multifocal leukoencephalopathy: report of three cases in HIV-negative hematological patients and review of literature. (18064459)
2008
30
The emergence of progressive multifocal leukoencephalopathy (PML) in rheumatic diseases. (18191544)
2008
31
The 50th birthday of progressive multifocal leukoencephalopathy: New insights into pathogenesis. (18991349)
2008
32
Improvement of progressive multifocal leukoencephalopathy after cidofovir therapy in a patient with a destructive polyarthritis. (17297588)
2007
33
Progressive multifocal leukoencephalopathy in an AIDS patient. (17493905)
2007
34
Characterization of JC virus in cerebrospinal fluid from HIV-1 infected patients with progressive multifocal leukoencephalopathy: insights into viral pathogenesis and disease prognosis. (17849317)
2007
35
Progressive multifocal leukoencephalopathy in rheumatic diseases: evolving clinical and pathologic patterns of disease. (17599729)
2007
36
JC Virus Infection After Transplantation: Beyond the Classic Progressive Multifocal Leukoencephalopathy? (21960781)
2007
37
Remission of progressive multifocal leukoencephalopathy and primary central nervous system lymphoma in an HIV-infected patient. (17539934)
2007
38
Progressive multifocal leukoencephalopathy. (18808984)
2007
39
Chimeric immune receptors (CIRs) specific to JC virus for immunotherapy in progressive multifocal leukoencephalopathy (PML). (17660502)
2007
40
Progressive multifocal leukoencephalopathy in HIV-2-infected patient. (16816570)
2006
41
Natalizumab and progressive multifocal leukoencephalopathy: highlights of the International Workshop on JC Virus/PML and Multiple Sclerosis, June 3-4, 2005, Philadelphia PA. (16400315)
2005
42
AIDS-related progressive multifocal leukoencephalopathy. (16047070)
2005
43
Elevation of myoinositol is associated with disease containment in progressive multifocal leukoencephalopathy. (15365144)
2004
44
Progressive multifocal leukoencephalopathy in an adult patient with ICF syndrome. (14675617)
2004
45
Early events in the life cycle of JC virus as potential therapeutic targets for the treatment of progressive multifocal leukoencephalopathy. (12709869)
2003
46
Clinical epidemiology and survival of progressive multifocal leukoencephalopathy in the era of highly active antiretroviral therapy: data from the Italian Registry Investigative Neuro AIDS (IRINA). (12709872)
2003
47
Low prevalence of progressive multifocal leukoencephalopathy in India and Africa: is there a biological explanation? (12709874)
2003
48
Reduced concentrations of HIV-RNA and TNF-alpha coexist in CSF of AIDS patients with progressive multifocal leukoencephalopathy. (10449561)
1999
49
Progressive multifocal leukoencephalopathy in a 15-year-old boy with scleroderma and secondary amyloidosis. (9832587)
1998
50
An unusual course of progressive multifocal leukoencephalopathy in a patient with idiopathic CD4+ T lymphocytopenia. (9647312)
1998

Genetic Variations for Progressive Multifocal Leukoencephalopathy

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Expression for genes affiliated with Progressive Multifocal Leukoencephalopathy

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Progressive Multifocal Leukoencephalopathy

Search GEO for disease gene expression data for Progressive Multifocal Leukoencephalopathy.

Pathways for genes affiliated with Progressive Multifocal Leukoencephalopathy

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37NCBI BioSystems Database
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Pathways related to Progressive Multifocal Leukoencephalopathy according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Hide members
10.2ADA, PNP

Compounds for genes affiliated with Progressive Multifocal Leukoencephalopathy

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Sources:
44Novoseek, 24HMDB, 11DrugBank, 59Tocris Bioscience, 28IUPHAR, 49PharmGKB
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Compounds related to Progressive Multifocal Leukoencephalopathy according to GeneCards/GeneDecks:

(show all 26)
idCompoundScoreTop Affiliating Genes
1deoxyinosine44 2411.6PNP, ADA
29-(beta-d-arabinofuranosyl)guanine4410.6ADA, PNP
3nelarabine44 1111.5ADA, PNP
4cordycepin4410.5ADA, TAT
52,3-dideoxyadenosine4410.5ADA, PNP
6pentostatin44 59 28 1113.5ADA, PNP
72,5-oligoadenylate4410.4CT49, TAT, ADA
8ribavirin44 49 1112.4LBR, PNP, ADA
9tuberculin4410.4LBR, ADA
10guanosine44 11 2412.3MECP2, TAT, PNP, ADA
11deoxyadenosine44 2411.3ADA, PNP
12neopterin4410.3LBR, CD9, ADA
13didanosine44 1111.3ADA, PNP, CD9, LBR
14vincristine44 49 1112.3ADA, TAT, ITGA4
15pentoxifylline44 1111.3LBR, ITGA4, CD9, TAT
16rantes4410.3CD9, ITGA4, LBR
17zidovudine44 1111.3LBR, CD9, TAT, ADA
18purine44 2411.2ADA, PNP, CD9, LBR
19mdms4410.2CD9, TAT
20adenosine28 11 2412.1PNP, ADA
21gold4410.0TAT, CD9, LBR
22alanine449.9CT49, MECP2, CD9, TAT, PNP, ADA
23cysteine449.8CT49, LBR, CD9, TAT, PNP, ADA
24retinoic acid44 2410.8CT49, LBR, ITGA4, CD9, TAT, ADA
25oligonucleotide449.8PURA, CT49, MECP2, CD9, TAT
26tyrosine449.2PDCD1, LBR, ITGA4, CD9, TAT, PNP

GO Terms for genes affiliated with Progressive Multifocal Leukoencephalopathy

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16Gene Ontology
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Cellular components related to Progressive Multifocal Leukoencephalopathy according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1external side of plasma membraneGO:0098979.9ADA, CD9, ITGA4, PDCD1

Biological processes related to Progressive Multifocal Leukoencephalopathy according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1purine-containing compound salvageGO:04310110.2ADA, PNP
2positive regulation of alpha-beta T cell differentiationGO:0466389.9ADA, PNP

Products for genes affiliated with Progressive Multifocal Leukoencephalopathy

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Sources for Progressive Multifocal Leukoencephalopathy

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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
61UMLS via Orphanet