Demyelination as a harbinger of lymphoma: A case report and review of primary central nervous system lymphoma preceded by multifocal sentinel demyelination

Mark D. Kvarta, Deva Sharma, Rudolph J. Castellani, Robert E. Morales, Stephen G. Reich, Amy S. Kimball, Robert K. Shin*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

31 Scopus citations

Abstract

Background: Primary central nervous system lymphoma (PCNSL) may rarely be preceded by "sentinel demyelination," a pathologic entity characterized by histologically confirmed demyelinating inflammatory brain lesions that mimic multiple sclerosis (MS) or acute disseminated encephalomyelitis (ADEM). Interpreting the overlapping radiologic and clinical characteristics associated with each of these conditions-contrast-enhancing demyelination of white matter and relapsing and remitting steroid-responsive symptoms respectively-can be a significant diagnostic challenge. Case presentation: We describe a 57-year-old woman with an unusual clinical course who presented with multi-focal enhancing white matter lesions demonstrated to be inflammatory demyelination by brain biopsy. Despite a good initial response to steroids and rituximab for treatment of presumed tumefactive multiple sclerosis, the patient's condition rapidly deteriorated, and a repeat brain biopsy six months later was consistent with a diagnosis of diffuse large B-cell lymphoma. Conclusions: Early clinical suspicion for PCNSL and awareness that biopsied lesions may initially show sentinel demyelination suggestive of alternate diagnoses may be essential for early initiation of appropriate therapies and mitigation of disease progression. Clinical, pathophysiological, and diagnostic aspects of sentinel demyelination and PCNSL are discussed.

Original languageEnglish (US)
Article number72
JournalBMC Neurology
Volume16
Issue number1
DOIs
StatePublished - May 21 2016

Keywords

  • Demyelination
  • Multiple Sclerosis
  • Pre-operative steroids
  • Primary CNS Lymphoma

ASJC Scopus subject areas

  • Clinical Neurology

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