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Progressive multifocal leukoencephalopathy and other forms of JC virus disease

Bruce J Brew1, Nicholas W S Davies, Paola Cinque

  • 1Department of Neurology and St Vincent's Center for Applied Medical Research, St Vincent's Hospital and University of New South Wales, Victoria Street, Sydney, NSW 2010, Australia. b.brew@unsw.edu.au

Nature Reviews. Neurology
|December 7, 2010
PubMed

Insights

Progressive multifocal leukoencephalopathy (PML), a brain disease caused by JC virus (JCV), requires prompt intervention. Reconstituting the immune system is key for managing PML and improving patient prognosis.

Area of Science:

  • Neurology
  • Virology
  • Immunology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating brain disease.
  • PML is caused by the JC virus (JCV) and typically occurs in immunocompromised individuals.
  • High mortality rates (20-50% within 3 months) necessitate urgent management.

Purpose of the Study:

  • To critically review controversies surrounding JCV infection.
  • To provide practical management guidelines for PML.
  • To highlight the importance of prompt intervention in PML cases.

Main Methods:

  • Review of existing literature on PML and JCV infection.
  • Analysis of diagnostic criteria including neurological presentation, MRI, and CSF JCV DNA.
  • Discussion of therapeutic strategies focusing on immune reconstitution.

Main Results:

  • Diagnosis relies on clinical, radiological, and laboratory findings.
  • Immune system reconstitution is the primary treatment strategy.
  • Suspension or reduction of immunosuppressive therapy is crucial.
  • Plasma exchange may be considered for long half-life therapies.

Conclusions:

  • Prompt diagnosis and intervention are essential for PML management.
  • Restoring immune function offers the best prognosis.
  • Careful management is needed to balance immune recovery and potential disease worsening.

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