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Progressive multifocal leukoencephalopathy in multiple sclerosis.

Joshua J Chalkley1, Joseph R Berger

  • 1Department of Neurology, University of Kentucky College of Medicine, 740 S. Limestone St., Room L-445, Lexington, KY, 40536-0284, USA, josh.chalkley@uky.edu.

Current Neurology and Neuroscience Reports
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Summary
This summary is machine-generated.

Progressive multifocal leukoencephalopathy (PML) is a rare brain disease that has seen increased interest due to its association with certain therapies, particularly natalizumab for multiple sclerosis (MS). Risk mitigation strategies are available for natalizumab-associated PML.

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Area of Science:

  • Neuroimmunology
  • Infectious Diseases
  • Demyelinating Diseases

Background:

  • Acquired immunodeficiency syndrome (AIDS) increased interest in progressive multifocal leukoencephalopathy (PML).
  • A second wave of PML cases emerged with biological agents like natalizumab and efalizumab.
  • Natalizumab, used for multiple sclerosis (MS), is associated with a significant number of PML cases.

Purpose of the Study:

  • To review the pathogenesis, clinical manifestations, and treatment of PML, especially in the context of therapeutic agents.
  • To highlight the risks, mitigation strategies, and treatment of natalizumab-associated PML and PML immune reconstitution inflammatory syndrome.
  • To compare natalizumab-associated PML with HIV-related PML.

Main Methods:

  • Review of reported cases of natalizumab-associated PML.
  • Analysis of risk factors and clinical presentation.
  • Comparison of PML characteristics between different etiologies.

Main Results:

  • Approximately 400 cases of natalizumab-associated PML were reported from 2005 to August 2013.
  • Natalizumab-associated PML shows distinct differences from HIV-related PML, including higher incidence of radiographically isolated PML and cognitive disturbances.
  • Mortality for natalizumab-associated PML is substantially lower than for HIV-related PML.

Conclusions:

  • Practitioners must understand the risks of PML associated with MS therapies like natalizumab.
  • Risk mitigation strategies for natalizumab-associated PML are linked to therapy duration, JC virus seropositivity, and prior immunosuppression.
  • Effective management requires awareness of PML, its treatment, and associated syndromes.