MCID: PRG009
MIFTS: 45

Progressive Multifocal Leukoencephalopathy malady

Rare diseases, Infectious diseases, Neuronal diseases categories

Summaries for Progressive Multifocal Leukoencephalopathy

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NIH Rare Diseases:41 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 based summary: Progressive Multifocal Leukoencephalopathy, also known as leukoencephalopathy, progressive multifocal, is related to systemic lupus erythematosus and chronic lymphocytic leukemia. An important gene associated with Progressive Multifocal Leukoencephalopathy is IGHMBP2 (immunoglobulin mu binding protein 2), and among its related pathways is Cell adhesion molecules (CAMs). The compounds phorbol 12,13-dibutyrate and infliximab have been mentioned in the context of this disorder. Affiliated tissues include brain, t cells and kidney, and related mouse phenotypes are cellular and hematopoietic system.

Disease Ontology:9 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:42 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) is a rare and usually fatal viral disease characterized... more...

Aliases & Classifications for Progressive Multifocal Leukoencephalopathy

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Sources:
9Disease Ontology, 41NIH Rare Diseases, 42NINDS, 11DISEASES, 47Orphanet, 60UMLS, 43Novoseek, 27ICD9CM, 55SNOMED-CT, 33MeSH, 38NCIt, 34MESH via Orphanet, 61UMLS via Orphanet, 25ICD10
See all sources

Progressive Multifocal Leukoencephalopathy, Aliases & Descriptions:

Name: Progressive Multifocal Leukoencephalopathy 9 41 42 11 47
Leukoencephalopathy, Progressive Multifocal 41 60
Leukoencephalopathy Progressive Multifocal 43
 
Progressive Multifocal Leukoencephalitis 47
Pml 41


Classifications:

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


Characteristics (Orphanet epidemiological data):

47
progressive multifocal leukoencephalopathy:
Inheritance: Not applicable; Age of onset: All ages


External Ids:

Disease Ontology9 DOID:643
ICD9CM27 046.3
MeSH33 D007968
NCIt38 C26815
SNOMED-CT55 22255007
Orphanet47 217260
MESH via Orphanet34 D007968
UMLS via Orphanet61 C0023524
ICD1025 A81.2

Related Diseases for Progressive Multifocal Leukoencephalopathy

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



Diseases related to progressive multifocal leukoencephalopathy

Symptoms for Progressive Multifocal Leukoencephalopathy

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Drugs & Therapeutics for Progressive Multifocal Leukoencephalopathy

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Drug clinical trials:

Search ClinicalTrials for Progressive Multifocal Leukoencephalopathy

Search NIH Clinical Center for Progressive Multifocal Leukoencephalopathy

Genetic Tests for Progressive Multifocal Leukoencephalopathy

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Anatomical Context for Progressive Multifocal Leukoencephalopathy

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MalaCards organs/tissues related to Progressive Multifocal Leukoencephalopathy:

31
Brain, T cells, Kidney, Lung, B cells, Bone marrow, Bone, Liver, Spinal cord, Testes, Cortex, Heart

Animal Models for Progressive Multifocal Leukoencephalopathy or affiliated genes

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MGI Mouse Phenotypes related to Progressive Multifocal Leukoencephalopathy:

35
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053848.1PDCD1, ITGA4, PNP, LBR
2MP:00053978.0LBR, PNP, ITGA4, PDCD1
3MP:00053877.8LBR, PNP, ITGA4, PDCD1
4MP:00107687.6PDCD1, ITGA4, PNP, IGHMBP2, LBR

Publications for Progressive Multifocal Leukoencephalopathy

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Articles related to Progressive Multifocal Leukoencephalopathy:

(show top 50)    (show all 555)
idTitleAuthorsYear
1
Anti-JC virus antibody levels in serum or plasma further define risk of natalizumab-associated progressive multifocal leukoencephalopathy. (25273271)
2014
2
Progressive multifocal leukoencephalopathy in a patient with chronic lymphocytic leukaemia treated with alemtuzumab. (24403383)
2014
3
Progressive multifocal leukoencephalopathy in patients with sarcoidosis. (25267986)
2014
4
Partial recovery after severe immune reconstitution inflammatory syndrome in a multiple sclerosis patient with progressive multifocal leukoencephalopathy. (24341880)
2014
5
Efficacy of recombinant human interleukin 7 in a patient with severe lymphopenia-related progressive multifocal leukoencephalopathy. (25734144)
2014
6
Treatment of progressive multifocal leukoencephalopathy. (23345466)
2013
7
Progressive multifocal leukoencephalopathy in patients with multiple sclerosis. (23493158)
2013
8
Isolated cortical blindness without simultaneous neurological involvement in progressive multifocal leukoencephalopathy in a patient with human immune deficiency virus infection. (23514446)
2013
9
Progressive multifocal leukoencephalopathy with gastrointestinal disease in a pediatric kidney transplant recipient. (23902604)
2013
10
Progressive multifocal leukoencephalopathy: new concepts. (24141508)
2013
11
Unusual natalizumab-associated progressive multifocal leukoencephalopathy starting in the brainstem. (24276521)
2013
12
Progressive multifocal leukoencephalopathy and palliative care: a report of three cases and review of the literature. (22740610)
2013
13
Progressive multifocal leukoencephalopathy: clinical and molecular aspects. (21936015)
2012
14
Efficacy of mefloquine to progressive multifocal leukoencephalopathy initially presented with parkinsonism. (22209147)
2012
15
Brentuximab associated progressive multifocal leukoencephalopathy. (22472351)
2012
16
Natalizumab-associated progressive multifocal leukoencephalopathy. (22931329)
2012
17
The basis for modeling progressive multifocal leukoencephalopathy pathogenesis. (21499097)
2011
18
The pathogenesis of progressive multifocal leukoencephalopathy. (22204766)
2011
19
Infrequent detection of KI, WU and MC polyomaviruses in immunosuppressed individuals with or without progressive multifocal leukoencephalopathy. (21436884)
2011
20
Progressive multifocal leukoencephalopathy in liver transplant recipients: a case report and review of the literature. (21134241)
2011
21
Progressive multifocal leukoencephalopathy: can we reduce risk in patients receiving biological immunomodulatory therapies? (20818784)
2010
22
Treatment of progressive multifocal leukoencephalopathy and idiopathic CD4+ lymphocytopenia. (20961908)
2010
23
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
24
Progressive multifocal leukoencephalopathy in HIV-2 infection. Case report. (20452275)
2010
25
Incidence of progressive multifocal leukoencephalopathy in patients without HIV. (20938025)
2010
26
Detection of WU polyomavirus in cerebrospinal fluid specimen from a patient with AIDS and suspected progressive multifocal leukoencephalopathy. (19530941)
2009
27
Follow-up magnetic resonance imaging findings in patients with progressive multifocal leukoencephalopathy: evaluation of long-term survivors under highly active antiretroviral therapy. (19373535)
2009
28
Incidence, clinical presentation, and outcome of progressive multifocal leukoencephalopathy in HIV-infected patients during the highly active antiretroviral therapy era: a nationwide cohort study. (19007313)
2009
29
Progressive multifocal leukoencephalopathy--incidental finding in the forensic neuropathological examination. (19216217)
2009
30
WU and KI polyomaviruses in the brains of HIV-positive patients with and without progressive multifocal leukoencephalopathy. (19860559)
2009
31
Rearrangement of the JC virus regulatory region sequence in the bone marrow of a patient with rheumatoid arthritis and progressive multifocal leukoencephalopathy. (18989816)
2008
32
Progressive multifocal leukoencephalopathy in purine nucleoside phosphorylase deficiency. (16949240)
2007
33
Non-HIV-related progressive multifocal leukoencephalopathy (PML): a tertiary cancer center experience. (17238872)
2007
34
Inflammatory progressive multifocal leukoencephalopathy in human immunodeficiency virus-negative patients. (17328067)
2007
35
Central nervous system graft-versus-host disease: consider progressive multifocal leukoencephalopathy among the differential diagnoses. (17922040)
2007
36
Potential risk of progressive multifocal leukoencephalopathy with natalizumab therapy: possible interventions. (17296831)
2007
37
Multiple sclerosis, natalizumab therapy, and progressive multifocal leukoencephalopathy. (16914971)
2006
38
Progressive multifocal leukoencephalopathy: prolonged survival in patients treated with protease inhibitors and cidofovir: a case series. (16514320)
2006
39
Characterization of lymphocytic infiltrates in progressive multifocal leukoencephalopathy: co-localization of CD8(+) T cells with JCV-infected glial cells. (16798673)
2006
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
Successful outcome of progressive multifocal leukoencephalopathy in a renal transplant patient. (15816900)
2005
43
High anti-double-stranded DNA antibodies and progressive multifocal leukoencephalopathy in a patient with systemic lupus erythematosus. (15769818)
2005
44
Rearrangement of simian virus 40 regulatory region is not required for induction of progressive multifocal leukoencephalopathy in immunosuppressed rhesus monkeys. (15650162)
2005
45
Progressive multifocal leukoencephalopathy in transplant recipients. (15616809)
2005
46
Head tremor and progressive multifocal leukoencephalopathy in AIDS patients: report of two cases. (15830082)
2005
47
A prospective study demonstrates an association between JC virus-specific cytotoxic T lymphocytes and the early control of progressive multifocal leukoencephalopathy. (15215217)
2004
48
Progressive multifocal leukoencephalopathy in a patient with chronic myelogenous leukemia. (12802840)
2003
49
Search for human herpesvirus 6 DNA in cerebrospinal fluid from AIDS patients with progressive multifocal leukoencephalopathy. (12587078)
2003
50
Progressive multifocal leukoencephalopathy in AIDS: a clinicopathologic study and review of the literature. (8410079)
1993

Variations for Progressive Multifocal Leukoencephalopathy

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Expression for genes affiliated with Progressive Multifocal Leukoencephalopathy

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

Pathways for genes affiliated with Progressive Multifocal Leukoencephalopathy

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Pathways related to Progressive Multifocal Leukoencephalopathy according to GeneCards Suite gene sharing:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5PDCD1, ITGA4

Compounds for genes affiliated with Progressive Multifocal Leukoencephalopathy

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Compounds related to Progressive Multifocal Leukoencephalopathy according to GeneCards Suite gene sharing:

(show all 29)
idCompoundScoreTop Affiliating Genes
1phorbol 12,13-dibutyrate439.7LBR, ITGA4
2infliximab43 49 1211.7LBR, ITGA4
3zidovudine43 49 1211.6LBR, TAT
4lead439.6ITGA4, TAT
5vitamin-e439.6TAT, LBR
6mifepristone43 28 59 1212.6LBR, TAT
7vincristine43 49 1211.6TAT, ITGA4
8gold439.4TAT, LBR
9guanosine43 24 1211.4TAT, PNP
10uridine43 24 1211.4TAT, PNP
11didanosine43 49 1211.4PNP, LBR
12dithiothreitol439.4PNP, ITGA4
13ribavirin43 49 1211.4PNP, LBR
14inositol439.3LBR, TAT
15purine43 2410.2PNP, LBR
16adenine43 24 1211.2TAT, PNP
17pentoxifylline43 1210.2TAT, ITGA4, LBR
18rantes439.2LBR, ITGA4, TAT
19genistein43 28 59 2 24 1214.2TAT, ITGA4, LBR
20heparin43 28 24 1212.1LBR, ITGA4, TAT
21cycloheximide439.1LBR, ITGA4, TAT
22arsenite43 2410.0PNP, LBR
23dexamethasone43 49 28 1212.0TAT, ITGA4, LBR
24aspartate439.0TAT, ITGA4, PNP
25retinoic acid43 249.7TAT, ITGA4, LBR
26glucose438.4TAT, ITGA4, PNP, LBR
27serine438.3TAT, ITGA4, PNP, LBR
28cysteine438.2TAT, PNP, LBR
29tyrosine437.9TAT, PDCD1, ITGA4, PNP, LBR

GO Terms for genes affiliated with Progressive Multifocal Leukoencephalopathy

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Cellular components related to Progressive Multifocal Leukoencephalopathy according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1external side of plasma membraneGO:00098979.5PDCD1, ITGA4

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
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
45OMIM
46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet