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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
26:48

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Published on: July 4, 2007

Progressive multifocal leukoencephalopathy in a patient treated with natalizumab

Annette Langer-Gould1, Scott W Atlas, Ari J Green

  • 1Department of Neurology, Stanford University School of Medicine, Stanford, Calif, USA. annette1@stanford.edu

The New England Journal of Medicine
|June 11, 2005
PubMed

Insights

Progressive multifocal leukoencephalopathy (PML) developed in a multiple sclerosis patient on interferon beta-1a and natalizumab. Early PML lesions mimicked multiple sclerosis, but rapid progression and subsequent immune reconstitution inflammatory syndrome (IRIS) were observed.

Area of Science:

  • Neuroimmunology
  • Infectious Diseases
  • Neurology

Background:

  • Multiple sclerosis (MS) is a chronic autoimmune disease affecting the central nervous system.
  • Immunosuppressive therapies, including natalizumab, are used to manage MS.
  • Progressive multifocal leukoencephalopathy (PML) is a rare but serious opportunistic infection linked to certain MS treatments.

Observation:

  • A patient with MS developed PML during treatment with interferon beta-1a and natalizumab.
  • The initial PML lesion on MRI was indistinguishable from an MS lesion.
  • PML progressed rapidly despite treatment with corticosteroids, cidofovir, and intravenous immune globulin.

Findings:

  • The patient became quadriparetic, globally aphasic, and minimally responsive due to PML.
  • Immune-reconstitution inflammatory syndrome (IRIS) developed three months after natalizumab discontinuation.
  • Treatment with systemic cytarabine led to clinical improvement two months later.

Implications:

  • This case highlights the diagnostic challenge of early PML in MS patients.
  • It underscores the potential for IRIS following natalizumab cessation in PML patients.
  • Effective management strategies for PML and associated IRIS in MS patients require further investigation.

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