PML
MCID: PRG009
MIFTS: 57

Progressive Multifocal Leukoencephalopathy (PML) malady

Infectious diseases, Neuronal diseases categories

Summaries for Progressive Multifocal Leukoencephalopathy

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Sources:
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, 44Novoseek, 48Orphanet, 60UMLS, 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, Testes, Spinal cord, 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:00053909.8CD9, NFIX, PDCD1, CARTPT
2MP:00053889.6LBR, MECP2, IGHMBP2, POU3F1, CD9, ADA
3MP:00053849.2LBR, MECP2, ADA, SPIB, PURA, PNP
4MP:00053979.2LBR, ITGA4, CD9, PNP, SPIB, ADA
5MP:00053869.2LBR, NFIX, PURA, CARTPT, MECP2, FEZ1
6MP:00053789.1CD9, POU3F1, IGHMBP2, NFIX, MECP2, LBR
7MP:00107689.0ITGA4, ADA, IGHMBP2, NFIX, MECP2, LBR
8MP:00053878.9ITGA4, TICAM2, PURA, CARTPT, MECP2, PDCD1
9MP:00036318.9MECP2, FEZ1, PDCD1, PURA, NFIX, IGHMBP2

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
Prophylactic antiepileptic treatment reduces seizure frequency in natalizumab-associated progressive multifocal leukoencephalopathy. (24409198)
2014
2
Treatment of progressive multifocal leukoencephalopathy. (23345466)
2013
3
Presumptive Progressive Multifocal Leukoencephalopathy in Multiple Sclerosis after Natalizumab Therapy. (23323645)
2013
4
Progressive multifocal leukoencephalopathy developed 26 years after renal transplantation. (23253820)
2013
5
Frequent infection of cortical neurons by JC virus in patients with progressive multifocal leukoencephalopathy. (22157619)
2012
6
Progressive multifocal leukoencephalopathy in autoimmune diseases. (22281228)
2012
7
Smoldering myeloma presenting as progressive multifocal leukoencephalopathy: a case report. (22747665)
2012
8
Favourable outcome of progressive multifocal leukoencephalopathy with mefloquine treatment in combination with antiretroviral therapy in an HIV-infected patient. (22930304)
2012
9
Progressive multifocal leukoencephalopathy restricted to the posterior fossa in a patient with systemic lupus erythematosus. (22218525)
2011
10
Progressive multifocal leukoencephalopathy in transplant recipients. (21823157)
2011
11
Progressive multifocal leukoencephalopathy in an HIV patient receiving successful long-term HAART. (21445699)
2011
12
Progressive multifocal leukoencephalopathy: an unexpected complication of modern therapeutic monoclonal antibody therapies. (22082208)
2011
13
Progressive multifocal leukoencephalopathy (PML) after transplantation. (21235709)
2011
14
Progressive multifocal leukoencephalopathy in a patient without immunodeficiency. (21734181)
2011
15
Early onset of natalizumab-related progressive multifocal leukoencephalopathy. (21900356)
2011
16
JC virus latency in the brain and extraneural organs of patients with and without progressive multifocal leukoencephalopathy. (20610709)
2010
17
Progressive multifocal leukoencephalopathy in a patient without apparent immunosuppression. (20920200)
2010
18
Progressive multifocal leukoencephalopathy in patients on immunomodulatory therapies. (19719397)
2010
19
Progressive multifocal leukoencephalopathy and newer biological agents. (20925435)
2010
20
A case of progressive multifocal leukoencephalopathy in a patient treated with infliximab. (20722036)
2010
21
Clinicopathological study of early progressive multifocal leukoencephalopathy incidentally found in a schizophrenia patient. (19170897)
2009
22
Determinants of survival in progressive multifocal leukoencephalopathy. (19901246)
2009
23
Progressive multifocal leukoencephalopathy - experimental options. (19782910)
2009
24
Progressive multifocal leukoencephalopathy in HIV-1 infection. (19778765)
2009
25
A progressive neurologic disorder with multiple CNS lesions: a neuroimaging clinicopathologic correlation. Progressive multifocal leukoencephalopathy (PML). (18819185)
2008
26
Progressive multifocal leukoencephalopathy in a patient with idiopathic CD4+ cells deficit. (18998035)
2008
27
Infratentorial progressive multifocal leukoencephalopathy in a patient treated with fludarabine and rituximab. (18379739)
2008
28
Progressive multifocal leukoencephalopathy. (18657729)
2008
29
Progressive multifocal leukoencephalopathy. (18579021)
2008
30
Non-HIV-related progressive multifocal leukoencephalopathy (PML): a tertiary cancer center experience. (17238872)
2007
31
Frequency and phenotype of JC virus-specific CD8+ T lymphocytes in the peripheral blood of patients with progressive multifocal leukoencephalopathy. (17229701)
2007
32
Progressive multifocal leukoencephalopathy mimicking central nervous system lupus. (17924609)
2007
33
Mirtazapine in progressive multifocal leukoencephalopathy associated with polycythemia vera. (17674313)
2007
34
Characterization of lymphocytic infiltrates in progressive multifocal leukoencephalopathy: co-localization of CD8(+) T cells with JCV-infected glial cells. (16798673)
2006
35
Natalizumab and progressive multifocal leukoencephalopathy: migrating towards safe adhesion molecule therapy in multiple sclerosis. (16687056)
2006
36
Progression of progressive multifocal leukoencephalopathy despite treatment with beta-interferon. (16769975)
2006
37
Successful outcome of progressive multifocal leukoencephalopathy in a renal transplant patient. (15816900)
2005
38
Successful cidofovir therapy of progressive multifocal leukoencephalopathy preceding angioimmunoblastic T-cell lymphoma. (16334909)
2005
39
Progressive multifocal leukoencephalopathy after natalizumab therapy for Crohn's disease. (15947080)
2005
40
Childhood case of progressive multifocal leukoencephalopathy with improved clinical outcome. (15832618)
2005
41
A prospective study demonstrates an association between JC virus-specific cytotoxic T lymphocytes and the early control of progressive multifocal leukoencephalopathy. (15215217)
2004
42
JC virus early protein detection by immunohistochemistry in progressive multifocal leukoencephalopathy: a comparative study with in situ hybridization and polymerase chain reaction. (15581180)
2004
43
Progressive multifocal leukoencephalopathy: cidofovir therapy in three patients with underlying hematological disease. (12814108)
2003
44
Progressive multifocal leukoencephalopathy shortly after the diagnosis of follicular lymphoma in a patient treated with fludarabine. (12091141)
2002
45
JC virus load in progressive multifocal leukoencephalopathy: analysis of the correlation between the viral burden in cerebrospinal fluid, patient survival, and the volume of neurological lesions. (12032891)
2002
46
Fatal immune restoration disease in human immunodeficiency virus type 1-infected patients with progressive multifocal leukoencephalopathy: impact of antiretroviral therapy-associated immune reconstitution. (12410486)
2002
47
Progressive multifocal leukoencephalopathy and oligodendroglioma in a monkey co-infected by simian immunodeficiency virus and simian virus 40. (10965804)
2000
48
Analysis of the transcriptional control region in progressive multifocal leukoencephalopathy. (11031693)
2000
49
Detection of JC virus in cerebrospinal fluid (CSF) samples from patients with progressive multifocal leukoencephalopathy but not in CSF samples from patients with herpes simplex encephalitis, enteroviral meningitis, or multiple sclerosis. (9542955)
1998
50
Progressive multifocal leukoencephalopathy in AIDS: a clinicopathologic study and review of the literature. (8410079)
1993

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