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Favourable outcome of progressive multifocal leucoencephalopathy in two patients with dermatomyositis

S Vulliemoz1, F Lurati-Ruiz, F-X Borruat

  • 1Service de Neurologie, Hôpitaux Universitaires de Genève, Geneva, Switzerland.

Journal of Neurology, Neurosurgery, and Psychiatry
|August 18, 2006
PubMed

Insights

Progressive multifocal leukoencephalopathy (PML) outcomes improved in two patients with dermatomyositis. Treatments included stopping or tapering immunosuppressants, cytosine-arabinoside, and mirtazapine, suggesting potential combination therapy for PML.

Area of Science:

  • Neuroimmunology
  • Virology
  • Demyelinating Diseases

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a severe demyelinating disease caused by the JC virus (JCV) in immunosuppressed individuals.
  • PML typically has a poor prognosis, highlighting the need for effective treatment strategies.

Observation:

  • Two patients with PML and dermatomyositis experienced favorable outcomes.
  • Treatment varied, with one patient discontinuing immunosuppressants and the other tapering them.
  • Immunological responses, specifically JCV-specific CD8+ T cell responses, differed between patients.

Findings:

  • Both patients received cytosine-arabinoside; one also received mirtazapine, a 5HT2A receptor antagonist.
  • A strong JCV-specific CD8+ T cell response correlated with a better outcome in one patient.
  • Partial tapering of immunosuppressants combined with specific antiviral and receptor antagonist therapies showed promise.

Implications:

  • Combination therapy involving cytosine-arabinoside and mirtazapine may offer a viable treatment option for HIV-negative PML patients.
  • Understanding the role of T cell response in PML prognosis could guide therapeutic interventions.
  • Further research is warranted to explore the efficacy of this combination therapy in a larger cohort.

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