Bing and Neel Syndrome

Abstract

Introduction. We report the case of a Bing and Neel syndrome revealed by an isolated left ptosis. Case Report. a 57-year-old man was followed up since October 2003 for a typical Waldenström's macroglobulinemia. A first complete remission was obtained with chlorambucil. In August 2004, he relapsed. A second complete remission was obtained with RFC chemotherapy regimen (rituximab, fludarabine, and cyclophosphamide). In October 2009, the patient presented with an isolated left ptosis revealing a Bing and Neel syndrome. The diagnosis was suspected on MRI and confirmed by the detection in the CSF of a monoclonal IgM similar to the one found in the plasma. A quite good partial remission has been obtained after one course of RDHAP (rituximab, dexamethasone, cytarabine, and cisplatin) and 3 courses of RDHOx (rituximab, dexamethasone, cytarabine, and oxaliplatin), in addition to ten intrahectal chemotherapy injections. The treatment was followed by intensification and autologous stem cell transplantation. At D58, the patient died due to a septic shock. Conclusion. BNS is a rare and potentially treatable complication of WM. It should be considered in patients with neurologic symptoms and a history of WM.

Bing-Neel syndrome, a rare complication of Waldenstrom macroglobulinemia: analysis of 44 cases and review of the literature. A study on behalf of the French Innovative Leukemia Organization (FILO).

“…We found 33 cases published in the literature during the same period (1995-2014), [4][5][6][7][9][10][11][12][13] which are some of the 56 cases described since the first description of BNS in 1936 (Table 4). The patients' characteristics were similar: the median age at the time of BNS diagnosis was 65.5 years (range, 50-84), and the majority of patients were male (71%).…”

Bing–Neel Syndrome in a Patient with Waldenstrom's Macroglobulinemia: A Challenging Diagnosis in the Face of Normal Brain Imaging

“…Central nervous system (CNS) manifestations in patients with WM are usually a result of serum hyperviscosity [2]. In some patients, central neurological symptoms can be caused by direct infiltration of CNS by malignant cells, also known as Bing-Neel syndrome (BNS) [3,4]. Diagnosis of BNS is usually suspected in patients with WM who exhibit CNS symptoms and abnormal brain magnetic resonance imaging (MRI) findings, which can then be confirmed with cerebrospinal fluid (CSF) analysis and/or biopsy [5,6].…”