Case Report on Bing-Neel Syndrome: A Challenging Diagnosis

Abstract

Lymphoplasmacytic lymphoma/ Waldenström’s macroglobulinemia (LPL/WM) is a rare indolent B-cell lymphoproliferative disorder which is characterized by either with tumor infiltration or is due to the synthesis of monoclonal IgM. Bing-Neel syndrome is a rare neurologic complication of Waldenström’s macroglobulinemia, which is a result of infiltration of the central nervous system by clonal lymphoplasmacytes. We present a rare clinical case of a patient, who one year after the diagnosis of Waldenström’s macroglobulinemia, progressed with diverse neurologic presentation which included progressive bilateral deafness, vertigo, discoordination, low grade right lagophthalmos and also cerebrospinal fluid involvement. Based on the discovery of clonal B-cells in the cerebrospinal fluid by flow cytometry and magnetic resonance imaging, the diagnosis was made. Bing-Neel syndrome should be considered in patients with neurologic symptoms and a history of Waldenström’s macroglobulinemia, because it is a rare manifestation, which may occur at any time during the course of the disease and may be associated with poor outcome if not treated.