Progressive Multifocal Leukoencephalopathy [version 1; referees: 2 approved]
Progressive multifocal leukoencephalopathy (PML) is a devastating demyelinating disease with significant morbidity and mortality and no effective, targeted therapies. It is most often observed in association with abnormalities of cell-mediated immunity, in particular human immunodeficiency virus (HI...
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| Format: | Article |
| Language: | English |
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F1000 Research Ltd
2015-12-01
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| Series: | F1000Research |
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| Online Access: | http://f1000research.com/articles/4-1424/v1 |
| _version_ | 1818842346293297152 |
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| author | Laura Adang Joseph Berger |
| author_facet | Laura Adang Joseph Berger |
| author_sort | Laura Adang |
| collection | DOAJ |
| description | Progressive multifocal leukoencephalopathy (PML) is a devastating demyelinating disease with significant morbidity and mortality and no effective, targeted therapies. It is most often observed in association with abnormalities of cell-mediated immunity, in particular human immunodeficiency virus (HIV) infection, but also occurs in association with lymphoproliferative diseases, certain immunosuppressive and immunomodulatory regimens, and other conditions. The etiologic agent of PML is a small, ubiquitous polyomavirus, the JC virus (JCV, also known as JCPyV), for which at least 50% of the adult general population is seropositive. PML results when JCV replicates within cerebral oligodendrocytes and astrocytes, leading to oligodendrocyte death and demyelination. Unfortunately, no treatments have been convincingly demonstrated to be effective, though some have been employed in desperation; treatment otherwise includes attempts to restore any immune system defect, such as the withdrawal of the causative agent if possible, and general supportive care. |
| first_indexed | 2024-12-19T04:40:31Z |
| format | Article |
| id | doaj.art-82c53629b71244cc95210276c0e1ac77 |
| institution | Directory Open Access Journal |
| issn | 2046-1402 |
| language | English |
| last_indexed | 2024-12-19T04:40:31Z |
| publishDate | 2015-12-01 |
| publisher | F1000 Research Ltd |
| record_format | Article |
| series | F1000Research |
| spelling | doaj.art-82c53629b71244cc95210276c0e1ac772022-12-21T20:35:37ZengF1000 Research LtdF1000Research2046-14022015-12-01410.12688/f1000research.7071.17611Progressive Multifocal Leukoencephalopathy [version 1; referees: 2 approved]Laura Adang0Joseph Berger1Division of Neurology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, 19104, USADepartment of Neurology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, 19104, USAProgressive multifocal leukoencephalopathy (PML) is a devastating demyelinating disease with significant morbidity and mortality and no effective, targeted therapies. It is most often observed in association with abnormalities of cell-mediated immunity, in particular human immunodeficiency virus (HIV) infection, but also occurs in association with lymphoproliferative diseases, certain immunosuppressive and immunomodulatory regimens, and other conditions. The etiologic agent of PML is a small, ubiquitous polyomavirus, the JC virus (JCV, also known as JCPyV), for which at least 50% of the adult general population is seropositive. PML results when JCV replicates within cerebral oligodendrocytes and astrocytes, leading to oligodendrocyte death and demyelination. Unfortunately, no treatments have been convincingly demonstrated to be effective, though some have been employed in desperation; treatment otherwise includes attempts to restore any immune system defect, such as the withdrawal of the causative agent if possible, and general supportive care.http://f1000research.com/articles/4-1424/v1HIV Infection & AIDS: ClinicalInfectious Diseases of the Nervous SystemMultiple Sclerosis & Related DisordersNeuroimagingViral Infections (without HIV) |
| spellingShingle | Laura Adang Joseph Berger Progressive Multifocal Leukoencephalopathy [version 1; referees: 2 approved] F1000Research HIV Infection & AIDS: Clinical Infectious Diseases of the Nervous System Multiple Sclerosis & Related Disorders Neuroimaging Viral Infections (without HIV) |
| title | Progressive Multifocal Leukoencephalopathy [version 1; referees: 2 approved] |
| title_full | Progressive Multifocal Leukoencephalopathy [version 1; referees: 2 approved] |
| title_fullStr | Progressive Multifocal Leukoencephalopathy [version 1; referees: 2 approved] |
| title_full_unstemmed | Progressive Multifocal Leukoencephalopathy [version 1; referees: 2 approved] |
| title_short | Progressive Multifocal Leukoencephalopathy [version 1; referees: 2 approved] |
| title_sort | progressive multifocal leukoencephalopathy version 1 referees 2 approved |
| topic | HIV Infection & AIDS: Clinical Infectious Diseases of the Nervous System Multiple Sclerosis & Related Disorders Neuroimaging Viral Infections (without HIV) |
| url | http://f1000research.com/articles/4-1424/v1 |
| work_keys_str_mv | AT lauraadang progressivemultifocalleukoencephalopathyversion1referees2approved AT josephberger progressivemultifocalleukoencephalopathyversion1referees2approved |