Review of Progressive Multifocal Leukoencephalopathy and Natalizumab

@article{Aksamit2006ReviewOP,
  title={Review of Progressive Multifocal Leukoencephalopathy and Natalizumab},
  author={Allen Jr. Aksamit},
  journal={The Neurologist},
  year={2006},
  volume={12},
  pages={293-298}
}
  • A. Aksamit
  • Published 1 November 2006
  • Medicine
  • The Neurologist
Background:Progressive multifocal leukoencephalopathy (PML), a destructive demyelinating infection which lytically infects oligodendrocytes, has occurred in patients treated with natalizumab. Magnetic resonance imaging (MRI) scan imaging of the brain gives clues to diagnosis but is nonspecific in distinguishing multiple sclerosis from PML. Spinal fluid detection of JC virus is specific but incompletely sensitive. Associated immunosuppression is typically of the cell-mediated type but can be… 
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There is risk for severe acute infections and reactivation of latent infections, such as JC virus reactivation leading to PML, and it is critical to proceed cautiously when immune system modification strategies are being evaluated for fear of unleashing undesirable or even fatal diseases.
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Overall, the prognosis of PML has improved with much lower mortality but still severe neurological deficits, and the possibility remains to improve the JCV- specific immune response.
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Natalizumab has a favourable risk-benefit ratio in the treatment of relapsing-remitting multiple sclerosis, however, because of the potential risk of progressive multifocal leukoencephalopathy, patients must be carefully selected and specific protocols must be followed during the drug's administration.
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Findings on the pathogenesis of PML are reviewed and the encouraging case reports of novel treatments are reviewed, including immunomodulation and/or inhibition of viral replication.
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Screening for JCV in CSF in natalizumab-treated patients could help identify those at heightened risk for developing PML and discontinuing treatment in these patients may abort development of the clinical illness.
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Clinical manifestations are nonspecific and are typical of any demyelinating disease, although PML is more aggressive and has a poor prognosis, although there is no curative therapy.
Progressive Multifocal Leukoencephalopathy as the First Manifestation of Occult Sarcoidosis: Case Report and Review of the Literature
TLDR
This is the first published case of PML as the first manifestation of sarcoidosis in the absence of inflammatory hematologic involvement, and a 68-year-old woman without known immune suppression presented with mental status changes over several weeks.
Dual Therapy with Cidofovir and Mirtazapine for Progressive Multifocal Leukoencephalopathy in a Sarcoidosis Patient
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This case suggests that dual therapy with cidofovir and mirtazapine might be an effective treatment option in PML patients with sarcoidosis with rapidly progressive dementia and right hemiparesis.
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