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[A case of age-related Epstein-Barr virus-associated B-cell lymphoproliferative disorder which presented with

Yoshikatsu Noda1, Yoshihisa Ohtsuka, Naoko Yasui

  • 1Department of Neurology, Kobe University Hospital

Rinsho Shinkeigaku = Clinical Neurology
|April 13, 2011
PubMed

Insights

A rare Epstein-Barr virus-associated B-cell lymphoproliferative disorder affected the central nervous system in an elderly patient. Despite treatment, the condition progressed, leading to a fatal outcome and confirmed by autopsy findings.

Area of Science:

  • Neurology
  • Oncology
  • Infectious Diseases

Background:

  • Epstein-Barr virus (EBV) can cause various conditions, including lymphoproliferative disorders.
  • Central nervous system (CNS) involvement by EBV is uncommon, particularly in older adults.

Observation:

  • A 65-year-old man presented with malaise and disturbed consciousness.
  • Brain MRI revealed FLAIR hyperintensities in the basal ganglia, temporal lobe, frontal lobe, and brainstem.
  • Cerebrospinal fluid showed elevated EBV-DNA and positive EBV antibody titers.

Findings:

  • Initial treatment with acyclovir and steroids was ineffective, with progressive neurological decline.
  • Autopsy identified atypical B-cells in brain tissue.
  • Immunohistochemistry confirmed Epstein-Barr virus-encoded small RNA (EBER) in situ hybridization (ISH) positivity.

Implications:

  • This case highlights a rare instance of CNS involvement by age-related EBV-associated B-cell lymphoproliferative disorder.
  • The findings underscore the importance of considering EBV-driven lymphoproliferative disorders in elderly patients with unexplained neurological symptoms.
  • Further research is needed to understand the pathogenesis and develop effective treatments for this rare condition.

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