Progressive Multifocal Leukoencephalopathy with Balanced CD4/CD8 T-Cell Infiltration and Good Response to Mefloquine Treatment

Intern Med. 2016;55(12):1631-5. doi: 10.2169/internalmedicine.55.6051. Epub 2016 Jun 15.

Abstract

A 53-year-old man was admitted for sub-acute progressive dementia and Gerstmann syndrome. MRI demonstrated lesions in the white matter involving the left parietal lobe, accompanied by speckled or faint linear peripheral enhancement. Brain biopsy revealed JC virus infection in oligodendrocytes and balanced infiltration of CD8+ and CD4+ T lymphocytes. We diagnosed progressive multifocal leukoencephalopathy (PML) with controlled inflammation. The finding of CD4/CD8 T cells in the infected PML brain suggested therapeutically valuable immune system involvement, which we decided to preserve by withholding corticosteroids. We treated the patient with risperidone, cytarabine and mefloquine to suppress virus replication, but not with the corticosteroid that is conventionally used in inflammatory PML cases. The patient was discharged three months after admission, and one year later, his score on the Mini-Mental State Examination had recovered to 26/30, from 5/30 on admission.

Publication types

  • Case Reports

MeSH terms

  • Antiviral Agents / therapeutic use*
  • Brain Diseases / diagnosis
  • Brain Diseases / drug therapy*
  • Brain Diseases / virology
  • CD4-Positive T-Lymphocytes / pathology
  • Cytarabine / therapeutic use*
  • Humans
  • JC Virus / drug effects
  • Japan
  • Leukoencephalopathy, Progressive Multifocal / diagnosis
  • Leukoencephalopathy, Progressive Multifocal / drug therapy*
  • Leukoencephalopathy, Progressive Multifocal / virology*
  • Male
  • Mefloquine / therapeutic use*
  • Middle Aged
  • Risperidone / therapeutic use*
  • Treatment Outcome
  • Virus Replication / drug effects

Substances

  • Antiviral Agents
  • Cytarabine
  • Risperidone
  • Mefloquine