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Progressive multifocal leukoencephalopathy

MedGen UID:
7327
•Concept ID:
C0023524
•
Disease or Syndrome
Synonyms: Encephalitis, JC Polyomavirus; Encephalopathies, JC Polyomavirus; Encephalopathy, JC Polyomavirus; JC Polyomavirus Encephalitis; JC Polyomavirus Encephalopathy; Leukoencephalopathies, Progressive Multifocal; Leukoencephalopathy, Progressive Multifocal; Multifocal Leukoencephalopathies, Progressive; Multifocal Leukoencephalopathy, Progressive; Progressive Multifocal Leukoencephalopathies; Progressive Multifocal Leukoencephalopathy
SNOMED CT: Progressive multifocal leukoencephalopathy (22255007); Multifocal leukoencephalopathy (22255007); PML - Progressive multifocal leukoencephalopathy (22255007); PMLE - Progressive multifocal leukoencephalopathy (22255007)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016318
Orphanet: ORPHA217260

Definition

A progressive demyelination within the central nervous system associated with reactivation of a latent JC virus infection. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProgressive multifocal leukoencephalopathy
Follow this link to review classifications for Progressive multifocal leukoencephalopathy in Orphanet.

Professional guidelines

PubMed

Advances in Treatment of Progressive Multifocal Leukoencephalopathy.
Bernard-Valnet R, Koralnik IJ, Du Pasquier R
Ann Neurol 2021 Dec;90(6):865-873. Epub 2021 Sep 7 doi: 10.1002/ana.26198. PMID: 34405435Free PMC Article
Treatment of Viral Encephalitis.
Aksamit AJ Jr
Neurol Clin 2021 Feb;39(1):197-207. Epub 2020 Nov 7 doi: 10.1016/j.ncl.2020.09.011. PMID: 33223083
HIV-associated opportunistic CNS infections: pathophysiology, diagnosis and treatment.
Bowen LN, Smith B, Reich D, Quezado M, Nath A
Nat Rev Neurol 2016 Oct 27;12(11):662-674. doi: 10.1038/nrneurol.2016.149. PMID: 27786246

Recent clinical studies

Etiology

Progressive Multifocal Leukoencephalopathy: Pathogenesis, Diagnostic Tools, and Potential Biomarkers of Response to Therapy.
Schweitzer F, Laurent S, Cortese I, Fink GR, Silling S, Skripuletz T, Metz I, Wattjes MP, Warnke C
Neurology 2023 Oct 17;101(16):700-713. Epub 2023 Jul 24 doi: 10.1212/WNL.0000000000207622. PMID: 37487750Free PMC Article
Progressive multifocal leukoencephalopathy and the spectrum of JC virus-related disease.
Cortese I, Reich DS, Nath A
Nat Rev Neurol 2021 Jan;17(1):37-51. Epub 2020 Nov 20 doi: 10.1038/s41582-020-00427-y. PMID: 33219338Free PMC Article
Progressive Multifocal Leukoencephalopathy.
Grebenciucova E, Berger JR
Neurol Clin 2018 Nov;36(4):739-750. doi: 10.1016/j.ncl.2018.06.002. PMID: 30366552
Progressive Multifocal Leukoencephalopathy and Monoclonal Antibodies: A Review.
Bohra C, Sokol L, Dalia S
Cancer Control 2017 Oct-Dec;24(4):1073274817729901. doi: 10.1177/1073274817729901. PMID: 28975841Free PMC Article
Progressive multifocal leukoencephalopathy and other disorders caused by JC virus: clinical features and pathogenesis.
Tan CS, Koralnik IJ
Lancet Neurol 2010 Apr;9(4):425-37. doi: 10.1016/S1474-4422(10)70040-5. PMID: 20298966Free PMC Article

Diagnosis

Revisiting JC virus and progressive multifocal leukoencephalopathy.
Rocchi A, Sariyer IK, Berger JR
J Neurovirol 2023 Oct;29(5):524-537. Epub 2023 Sep 2 doi: 10.1007/s13365-023-01164-w. PMID: 37659983
Progressive multifocal leukoencephalopathy and the spectrum of JC virus-related disease.
Cortese I, Reich DS, Nath A
Nat Rev Neurol 2021 Jan;17(1):37-51. Epub 2020 Nov 20 doi: 10.1038/s41582-020-00427-y. PMID: 33219338Free PMC Article
Progressive multifocal leukoencephalopathy.
Zhai S, Brew BJ
Handb Clin Neurol 2018;152:123-137. doi: 10.1016/B978-0-444-63849-6.00010-4. PMID: 29604971
Progressive multifocal leukoencephalopathy.
Alstadhaug KB, Myhr KM, Rinaldo CH
Tidsskr Nor Laegeforen 2017 Dec 12;137(23-24) doi: 10.4045/tidsskr.16.1092. PMID: 29231623
PML diagnostic criteria: consensus statement from the AAN Neuroinfectious Disease Section.
Berger JR, Aksamit AJ, Clifford DB, Davis L, Koralnik IJ, Sejvar JJ, Bartt R, Major EO, Nath A
Neurology 2013 Apr 9;80(15):1430-8. doi: 10.1212/WNL.0b013e31828c2fa1. PMID: 23568998Free PMC Article

Therapy

Comparison of switching to 6-week dosing of natalizumab versus continuing with 4-week dosing in patients with relapsing-remitting multiple sclerosis (NOVA): a randomised, controlled, open-label, phase 3b trial.
Foley JF, Defer G, Ryerson LZ, Cohen JA, Arnold DL, Butzkueven H, Cutter G, Giovannoni G, Killestein J, Wiendl H, Smirnakis K, Xiao S, Kong G, Kuhelj R, Campbell N; NOVA study investigators
Lancet Neurol 2022 Jul;21(7):608-619. Epub 2022 Apr 25 doi: 10.1016/S1474-4422(22)00143-0. PMID: 35483387
Progressive multifocal leukoencephalopathy and the spectrum of JC virus-related disease.
Cortese I, Reich DS, Nath A
Nat Rev Neurol 2021 Jan;17(1):37-51. Epub 2020 Nov 20 doi: 10.1038/s41582-020-00427-y. PMID: 33219338Free PMC Article
Progressive multifocal leukoencephalopathy.
Zhai S, Brew BJ
Handb Clin Neurol 2018;152:123-137. doi: 10.1016/B978-0-444-63849-6.00010-4. PMID: 29604971
Progressive multifocal leukoencephalopathy.
Alstadhaug KB, Myhr KM, Rinaldo CH
Tidsskr Nor Laegeforen 2017 Dec 12;137(23-24) doi: 10.4045/tidsskr.16.1092. PMID: 29231623
Progressive Multifocal Leukoencephalopathy and Monoclonal Antibodies: A Review.
Bohra C, Sokol L, Dalia S
Cancer Control 2017 Oct-Dec;24(4):1073274817729901. doi: 10.1177/1073274817729901. PMID: 28975841Free PMC Article

Prognosis

Progressive multifocal leukoencephalopathy and the spectrum of JC virus-related disease.
Cortese I, Reich DS, Nath A
Nat Rev Neurol 2021 Jan;17(1):37-51. Epub 2020 Nov 20 doi: 10.1038/s41582-020-00427-y. PMID: 33219338Free PMC Article
Long-term safety of vedolizumab for inflammatory bowel disease.
Loftus EV Jr, Feagan BG, Panaccione R, Colombel JF, Sandborn WJ, Sands BE, Danese S, D'Haens G, Rubin DT, Shafran I, Parfionovas A, Rogers R, Lirio RA, Vermeire S
Aliment Pharmacol Ther 2020 Oct;52(8):1353-1365. Epub 2020 Sep 2 doi: 10.1111/apt.16060. PMID: 32876349Free PMC Article
Progressive multifocal leukoencephalopathy.
Zhai S, Brew BJ
Handb Clin Neurol 2018;152:123-137. doi: 10.1016/B978-0-444-63849-6.00010-4. PMID: 29604971
Progressive multifocal leukoencephalopathy and rheumatoid arthritis treatments.
Clavel G, Moulignier A, Semerano L
Joint Bone Spine 2017 Dec;84(6):671-675. Epub 2017 Mar 18 doi: 10.1016/j.jbspin.2017.03.002. PMID: 28323224
The safety of vedolizumab for ulcerative colitis and Crohn's disease.
Colombel JF, Sands BE, Rutgeerts P, Sandborn W, Danese S, D'Haens G, Panaccione R, Loftus EV Jr, Sankoh S, Fox I, Parikh A, Milch C, Abhyankar B, Feagan BG
Gut 2017 May;66(5):839-851. Epub 2016 Feb 18 doi: 10.1136/gutjnl-2015-311079. PMID: 26893500Free PMC Article

Clinical prediction guides

Drug-Induced Progressive Multifocal Leukoencephalopathy (PML): A Systematic Review and Meta-Analysis.
Rindi LV, Zaçe D, Braccialarghe N, Massa B, Barchi V, Iannazzo R, Fato I, De Maria F, Kontogiannis D, Malagnino V, Sarmati L, Iannetta M
Drug Saf 2024 Apr;47(4):333-354. Epub 2024 Feb 7 doi: 10.1007/s40264-023-01383-4. PMID: 38321317
Progressive Multifocal Leukoencephalopathy: Pathogenesis, Diagnostic Tools, and Potential Biomarkers of Response to Therapy.
Schweitzer F, Laurent S, Cortese I, Fink GR, Silling S, Skripuletz T, Metz I, Wattjes MP, Warnke C
Neurology 2023 Oct 17;101(16):700-713. Epub 2023 Jul 24 doi: 10.1212/WNL.0000000000207622. PMID: 37487750Free PMC Article
Progressive Multifocal Leukoencephalopathy Treated by Immune Checkpoint Inhibitors.
Boumaza X, Bonneau B, Roos-Weil D, Pinnetti C, Rauer S, Nitsch L, Del Bello A, Jelcic I, Sühs KW, Gasnault J, Goreci Y, Grauer O, Gnanapavan S, Wicklein R, Lambert N, Perpoint T, Beudel M, Clifford D, Sommet A, Cortese I, Martin-Blondel G; Immunotherapy for PML Study Group
Ann Neurol 2023 Feb;93(2):257-270. Epub 2022 Oct 17 doi: 10.1002/ana.26512. PMID: 36151879Free PMC Article
Pembrolizumab Treatment for Progressive Multifocal Leukoencephalopathy.
Cortese I, Muranski P, Enose-Akahata Y, Ha SK, Smith B, Monaco M, Ryschkewitsch C, Major EO, Ohayon J, Schindler MK, Beck E, Reoma LB, Jacobson S, Reich DS, Nath A
N Engl J Med 2019 Apr 25;380(17):1597-1605. Epub 2019 Apr 10 doi: 10.1056/NEJMoa1815039. PMID: 30969503
Progressive Multifocal Leukoencephalopathy and Monoclonal Antibodies: A Review.
Bohra C, Sokol L, Dalia S
Cancer Control 2017 Oct-Dec;24(4):1073274817729901. doi: 10.1177/1073274817729901. PMID: 28975841Free PMC Article

Recent systematic reviews

Drug-Induced Progressive Multifocal Leukoencephalopathy (PML): A Systematic Review and Meta-Analysis.
Rindi LV, Zaçe D, Braccialarghe N, Massa B, Barchi V, Iannazzo R, Fato I, De Maria F, Kontogiannis D, Malagnino V, Sarmati L, Iannetta M
Drug Saf 2024 Apr;47(4):333-354. Epub 2024 Feb 7 doi: 10.1007/s40264-023-01383-4. PMID: 38321317
Characteristics of Progressive Multifocal Leukoencephalopathy Associated With Sarcoidosis Without Therapeutic Immune Suppression.
McEntire CRS, Fletcher A, Toledano M, Epstein S, White E, Tan CS, Mao-Draayer Y, Banks SA, Aksamit AJ, Gelfand JM, Thakur KT, Anand P, Cortese I, Bhattacharyya S
JAMA Neurol 2023 Jun 1;80(6):624-633. doi: 10.1001/jamaneurol.2023.0841. PMID: 37093609Free PMC Article
Progressive multifocal leukoencephalopathy in patients with systemic lupus erythematosus: a systematic literature review.
Henegar CE, Eudy AM, Kharat V, Hill DD, Bennett D, Haight B
Lupus 2016 May;25(6):617-26. Epub 2016 Jan 6 doi: 10.1177/0961203315622819. PMID: 26743322
Natalizumab in multiple sclerosis: discontinuation, progressive multifocal leukoencephalopathy and possible use in children.
Vitaliti G, Matin N, Tabatabaie O, Di Traglia M, Pavone P, Lubrano R, Falsaperla R
Expert Rev Neurother 2015;15(11):1321-41. Epub 2015 Oct 29 doi: 10.1586/14737175.2015.1102061. PMID: 26513633
Immune reconstitution inflammatory syndrome in patients starting antiretroviral therapy for HIV infection: a systematic review and meta-analysis.
Müller M, Wandel S, Colebunders R, Attia S, Furrer H, Egger M; IeDEA Southern and Central Africa
Lancet Infect Dis 2010 Apr;10(4):251-61. doi: 10.1016/S1473-3099(10)70026-8. PMID: 20334848Free PMC Article

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