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Progressive multifocal leucoencephalopathy

N J Dalsgaard Hansen1, C Madsen, E Stenager

  • 1Odense University Hospital, Neurological Department, Denmark.

Italian Journal of Neurological Sciences
|December 1, 1996
PubMed
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Progressive multifocal leukoencephalopathy (PML) is a rare brain disease caused by JC virus (JCV) in immunocompromised individuals. While often fatal, some treatments like cytosine arabinoside show promise, and further research may yield effective therapies.

Area of Science:

  • Neuroscience
  • Virology
  • Immunology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare, demyelinating central nervous system disease.
  • It is caused by the JC virus (JCV), a neurotropic papovavirus.
  • PML predominantly affects immunocompromised individuals with impaired cellular immunity.

Purpose of the Study:

  • To review the occurrence, clinical presentation, and treatment of PML.
  • To highlight the increased incidence of PML in relation to HIV infection.
  • To discuss diagnostic approaches and potential therapeutic strategies.

Main Methods:

  • Literature review of PML cases and treatment outcomes.
  • Analysis of factors contributing to increased PML incidence.

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  • Discussion of diagnostic methods including immunological investigation and brain biopsy.

    • Main Results:

    • PML most commonly affects cerebral hemispheres, cerebellum, and brain stem.
    • Increased use of immunosuppressive therapies and HIV/AIDS have led to a rise in PML cases.
    • Cytosine arabinoside has shown favorable results; AZT/zidovudine may help in HIV-infected patients.

    Conclusions:

    • PML remains a serious condition, often rapidly progressive and fatal.
    • Early diagnosis through immunological investigation is crucial.
    • Further research into pathogenesis, particularly in HIV-infected individuals, is needed for effective therapeutic strategies.