Abstract
Progressive multifocal leukoencephalopathy (PML) is a rare, myelin-damaging disease of the central nervous system (CNS) in a setting of immunosuppression that is superimposed by concurrent autoimmune diseases such as multiple sclerosis and AIDS or simultaneous administration of immune modulatory monoclonal antibody drugs such as natalizumab. The causative agent is a Polyomavirus known as John Cunningham (JC) virus (JCV) that affects oligodendrocytes and astrocytes resulting in focal, extensive and progressive demyelination across the brain. The pathogenesis of JCV latent and active infection is yet to be fully understood despite significant medical research. To date, no therapeutic intervention has been very effective in addressing the health implications of PML. In this article, we review the current knowledge on the life cycle of JCV, pathogenesis of PML, highlight important tools in the diagnosis, potential targets for management and therapeutic intervention of PML.
Highlights
Progressive multifocal leukoencephalopathy (PML) is a rare, myelin-degrading disease of the central nervous system (CNS) caused by infection of oligodendrocytes and astrocytes by a Polyomavirus known as John Cunningham (JC) virus (JCV), whose name was taken after the first patient in which the virus was first isolated [1]
Diagnosis of progressive multifocal leukoencephalopathy A combination of clinical signs, laboratory tests, histopathological findings and diagnostic imaging techniques are employed to arrive at a definitive diagnosis of PML
Detection and identification of JC viral particle, protein and or DNA fragments in brain tissue samples and or cerebrospinal fluid polymerase chain reaction (CSF-PCR) for JC virus DNA could serve as a definitive diagnosis of active JCV infection [1,66]
Summary
Progressive multifocal leukoencephalopathy (PML) is a rare, myelin-degrading disease of the central nervous system (CNS) caused by infection of oligodendrocytes and astrocytes by a Polyomavirus known as John Cunningham (JC) virus (JCV), whose name was taken after the first patient in which the virus was first isolated [1]. PML is been associated with numerous autoimmune and inflammatory diseases such as multiple sclerosis (MS) [9], acquired immune deficiency syndrome (AIDS) [16,17,18,19,20,21], lymphocytic leukemia [22], or during organ transplant co-administration of immunosuppressant drugs.
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
