Bing-Neel syndrome: an illustrative case and a comprehensive review of the published literature.
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.
Malkani
Roneil G
RG
Department of Neurology, Northwestern University, Feinberg School of Medicine, 710 North Lake Shore Dr., Abbott Hall Room 1123, Chicago, IL 60611, USA.
Tallman
Martin
M
Gottardi-Littell
Numa
N
Karpus
William
W
Marszalek
Laura
L
Variakojis
Daina
D
Kaden
Bruce
B
Walker
Matthew
M
Levy
Robert M
RM
Raizer
Jeffrey J
JJ
D018951:Antigens, CD20 / Q000378:metabolism; D005260:Female; D005434:Flow Cytometry / Q000379:methods; D006261:Headache / Q000150:complications* / Q000175:diagnosis; D006801:Humans; D017493:Leukocyte Common Antigens / Q000378:metabolism; D008214:Lymphocytes / Q000378:metabolism / Q000473:pathology; D016239:MEDLINE / Q000706:statistics & numerical data; D008279:Magnetic Resonance Imaging / Q000379:methods; D008875:Middle Aged; D019636:Neurodegenerative Diseases / Q000150:complications* / Q000175:diagnosis; D008258:Waldenstrom Macroglobulinemia / Q000150:complications* / Q000175:diagnosis
D018951:Antigens, CD20; D017493:Leukocyte Common Antigens