A Punctate Magnetic Resonance Imaging Pattern in a Patient with Systemic Lupus Erythematosus Is an Early Sign of Progressive Multifocal Leukoencephalopathy: A Clinicopathological Study

Intern Med. 2018 Sep 15;57(18):2727-2734. doi: 10.2169/internalmedicine.0696-17. Epub 2018 Apr 27.

Abstract

A 37-year-old woman with systemic lupus erythematosus presented with gait disturbance and cognitive dysfunction. Brain magnetic resonance imaging (MRI) revealed small, punctate, T2-/fluid-attenuated inversion recovery-hyperintense and T1-hypointense lesions without gadolinium enhancement, which is atypical for progressive multifocal leukoencephalopathy (PML). On a pathological examination of biopsied brain tissues, JC virus-infected cells were hardly detected via immunohistochemistry but were certainly detected via in situ hybridization, conclusively verifying the PML diagnosis. After tapering off the immunosuppressant and mefloquine administration, the MRI findings revealed gradual improvement, and she has been stable for over 18 months. A punctate MRI pattern is not specific to natalizumab-associated PML but may be a ubiquitous early sign useful for the early diagnosis of PML.

Keywords: early diagnosis; mefloquine; progressive multifocal leukoencephalopathy (PML); punctate pattern, pathological examination; systemic lupus erythematosus (SLE).

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biopsy
  • Brain / diagnostic imaging
  • Brain / pathology
  • Brain / virology
  • Female
  • Humans
  • Immunologic Factors / adverse effects
  • Immunologic Factors / therapeutic use
  • Immunosuppressive Agents
  • In Situ Hybridization
  • JC Virus / isolation & purification
  • Leukoencephalopathy, Progressive Multifocal / diagnostic imaging*
  • Leukoencephalopathy, Progressive Multifocal / pathology*
  • Lupus Erythematosus, Systemic / diagnostic imaging*
  • Lupus Erythematosus, Systemic / drug therapy
  • Lupus Erythematosus, Systemic / pathology*
  • Magnetic Resonance Imaging*
  • Mefloquine / adverse effects
  • Mefloquine / therapeutic use
  • Natalizumab / adverse effects
  • Natalizumab / therapeutic use

Substances

  • Immunologic Factors
  • Immunosuppressive Agents
  • Natalizumab
  • Mefloquine