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Favourable outcome of progressive multifocal leucoencephalopathy in two patients with dermatomyositis
  1. S Vulliemoz1,
  2. F Lurati-Ruiz2,
  3. F-X Borruat3,
  4. J Delavelle4,
  5. I J Koralnik5,
  6. T Kuntzer6,
  7. J Bogousslavsky6,
  8. F Picard1,
  9. T Landis1,
  10. R A Du Pasquier6
  1. 1Service de Neurologie, Hôpitaux Universitaires de Genève, Geneva, Switzerland
  2. 2Service d’ Immunologie et allergologie, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland
  3. 3Unité de Neuro-ophtalmologie, Centre Hospitalier Universitaire Vaudois, Lausanne
  4. 4Unité de Neuro-radiologie, Hôpitaux Universitaires de Genève
  5. 5HIV/Neurology Center, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA
  6. 6Service de Neurologie, Centre Hospitalier Universitaire Vaudois
  1. Correspondence to:
 R A Du Pasquier
 Service de Neurologie and Service d’Immunologie, Centre Hospitalier Universitaire Vaudois, BT-02 1011 Lausanne, Switzerland; renaud.Du-Pasquier{at}chuv.ch

Abstract

Progressive multifocal leucoencephalopathy (PML), a demyelinating disease caused by the JC virus (JCV), occurs in immunosuppressed patients and carries a poor prognosis. A favourable outcome is reported in two patients with PML and dermatomyositis. Immunosuppressive drugs were stopped in patient 1 but could only be partially tapered in patient 2. The JCV-specific CD8+ T cell response was strong in patient 1 and weak in patient 2. Both were treated with cytosine-arabinoside, and patient 2 was also treated with mirtazapine, a 5HT2A receptor antagonist. Combination of these drugs might be helpful to treat HIV-negative patients with PML.

  • Ara-C, cytosine-arabinoside
  • JCV, JC virus
  • MRI, magnetic resonance imaging
  • PCR, polymerase chain reaction
  • PML, progressive multifocal leucoencephalopathy

https://doi.org/10.1136/jnnp.2006.092353

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    Footnotes

    • This work was supported in part by grants from the Swiss National Foundation (FN 3200BO-104262 and PP00B-106716) and from the Swiss Society for Multiple Sclerosis to RADP, and grants R01 NS/AI 041198 and 047029 from the National Institutes of Health, USA to IJK.

    • Competing interests: None declared.

    • Informed consent was obtained from the patients described in this report.