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Multifocal Electroretinograms
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[Progressive multifocal leukoencephalopathy].

Antoine Moulignier1, Augustin Lecler2, Cédric Lamirel3

  • 1Service de neurologie.

La Revue Du Praticien
|December 5, 2018
PubMed
Summary
This summary is machine-generated.

Progressive multifocal leukoencephalopathy (PML) is a fatal CNS disease caused by JC virus reactivation during immunosuppression. Current treatment focuses on restoring immune response, as no specific effective therapy exists.

Keywords:
Encéphalopathie

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Area of Science:

  • Neurology
  • Virology
  • Immunology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease of the central nervous system (CNS).
  • It is caused by the human polyomavirus JC (JCV), which establishes a latent infection.
  • Reactivation of JCV, often due to immunosuppression, leads to PML and other CNS complications.

Purpose of the Study:

  • To provide a comprehensive overview of Progressive Multifocal Leukoencephalopathy (PML).
  • To discuss the role of JC virus (JCV) and immunosuppression in PML pathogenesis.
  • To outline diagnostic approaches and current therapeutic strategies for PML.

Main Methods:

  • Review of existing literature on PML.
  • Analysis of JCV reactivation mechanisms under immunosuppressive conditions.
  • Summary of diagnostic criteria including MRI, CSF analysis, and neuropathology.
  • Evaluation of treatment strategies aimed at immune reconstitution.

Main Results:

  • PML is a lethal CNS disease resulting from JC virus reactivation.
  • Immunosuppression, including HIV, monoclonal antibody therapy, and transplantation, is a critical factor.
  • Clinical manifestations vary based on lesion location.
  • Diagnosis relies on neuroimaging, JCV detection in cerebrospinal fluid, and potentially brain biopsy.

Conclusions:

  • PML is a devastating opportunistic infection of the CNS.
  • Effective treatments are lacking, emphasizing the importance of managing underlying immunosuppression.
  • Restoring the host immune response is the primary therapeutic goal for PML.