Abstract
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.
Highlights
Progressive multifocal leukoencephalopathy (PML), a typically rapidly progressive, potentially fatal neurologic syndrome, was first described in 1958 as a complication of chronic lymphocytic leukemia and Hodgkin’s disease[1]
Following the acquired immunodeficiency syndrome (AIDS) pandemic and with newer immunomodulatory therapies, such as natalizumab, that predispose to the development of PML, the incidence of the disease has increased substantially
Even among the immunocompromised, only a small subset of infected patients develop PML, as the development of this syndrome requires a complex series of events: the virus must be transformed to the prototype virus, seed the brain, and avoid neuro-immunosurveillance and clearance
Summary
Progressive multifocal leukoencephalopathy (PML), a typically rapidly progressive, potentially fatal neurologic syndrome, was first described in 1958 as a complication of chronic lymphocytic leukemia and Hodgkin’s disease[1]. Keywords Progressive multifocal leukoencephalopathy , demyelination , JC virus , immunocompromised , highly active antiretroviral therapy Invited Reviewers version 1 published 10 Dec 2015
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