Journal of Neuro-Oncology

, Volume 96, Issue 3, pp 301–312

Bing–Neel syndrome: an illustrative case and a comprehensive review of the published literature

  • Roneil G. Malkani
  • Martin Tallman
  • Numa Gottardi-Littell
  • William Karpus
  • Laura Marszalek
  • Daina Variakojis
  • Bruce Kaden
  • Matthew Walker
  • Robert M. Levy
  • Jeffrey J. Raizer
Topic Review

DOI: 10.1007/s11060-009-9968-3

Cite this article as:
Malkani, R.G., Tallman, M., Gottardi-Littell, N. et al. J Neurooncol (2010) 96: 301. doi:10.1007/s11060-009-9968-3

Abstract

Waldenstrom’s macroglobulinemia (WM) is a chronic lymphoproliferative disorder within the spectrum of lymphoplasmacytic lymphoma characterized by proliferation of plasma cells, small lymphocytes, and plasmacytoid lymphocytes. Central nervous system involvement is very rare (Bing–Neel [BN] syndrome). We present the case of a 62-year-old woman previously diagnosed with WM who presented with Bing–Neel syndrome and review the published literature which consists of only case reports. We performed a Medline search using the terms “Waldenstrom’s macroglobulinemia and central nervous system” and “Bing–Neel” collecting data on presentation, evaluation, treatment, and outcome and summarizing these findings in the largest pooled series to date. Central nervous system manifestations are localization related. Serum laboratory testing reflects systemic disease. Cerebrospinal fluid analysis may show lymphocytic pleocytosis, elevated protein, and IgM kappa or lambda light chain restriction; cytology results are variable. Imaging is frequently abnormal. Biopsy confirms the diagnosis. Treatment data are limited, but responses are seen with radiation and/or chemotherapy. BN syndrome is a very rare complication of WM that should be considered in patients with neurologic symptoms and a history of WM. Treatment should be initiated as responses do occur that may improve quality of life and extend it when limited or no active systemic disease is present.

Keywords

Bing–Neel syndromeWaldenstrom’s macroglobulinemiaRituximabCentral nervous systemNeurologic complications

Copyright information

© Springer Science+Business Media, LLC. 2009

Authors and Affiliations

  • Roneil G. Malkani
    • 1
  • Martin Tallman
    • 2
  • Numa Gottardi-Littell
    • 3
  • William Karpus
    • 3
  • Laura Marszalek
    • 3
  • Daina Variakojis
    • 3
  • Bruce Kaden
    • 6
  • Matthew Walker
    • 4
  • Robert M. Levy
    • 5
  • Jeffrey J. Raizer
    • 1
  1. 1.Department of NeurologyNorthwestern University, Feinberg School of MedicineChicagoUSA
  2. 2.Section of Hematology/Oncology, Department of MedicineNorthwestern University, Feinberg School of MedicineChicagoUSA
  3. 3.Department of PathologyNorthwestern University, Feinberg School of MedicineChicagoUSA
  4. 4.Department of RadiologyNorthwestern University, Feinberg School of MedicineChicagoUSA
  5. 5.Department of NeurosurgeryNorthwestern University, Feinberg School of MedicineChicagoUSA
  6. 6.Department of Oncology-HematologyAdvocate Lutheran General HospitalPark RidgeUSA