Lymphomatosis cerebri masquerading as the Marburg variant of multiple sclerosis

Mult Scler Relat Disord. 2020 Nov:46:102488. doi: 10.1016/j.msard.2020.102488. Epub 2020 Sep 3.

Abstract

Lymphomatosis cerebri (LC) is a rare variant of primary central nervous system lymphoma with few cases reported. Here, we describe the case of a patient with clinical presentation, imaging, and biopsy in keeping with aggressive multiple sclerosis (MS) such as that in Marburg variant. He deteriorated clinically over 9 months. Post-mortem examination yielded a diagnosis of LC with B-cell lymphoma. LC is notoriously difficult to diagnose, as it can present in various ways and biopsy of unaffected areas will be non-diagnostic. In our case, diagnosis was made more challenging by the patient's dramatic response to treatment with steroids and cyclophosphamide.

Keywords: Lymphomatosis cerebri; Marburg; Multiple sclerosis; Neuropathology; Primary CNS lymphoma.

Publication types

  • Case Reports
  • Letter

MeSH terms

  • Biopsy
  • Brain Neoplasms*
  • Central Nervous System Neoplasms*
  • Cyclophosphamide / therapeutic use
  • Humans
  • Male
  • Multiple Sclerosis* / diagnostic imaging
  • Multiple Sclerosis* / drug therapy

Substances

  • Cyclophosphamide