Syndrome de Bing-Neel révélateur d’une maladie de Waldenström : étude d’un cas et revue de la littérature

Introduction

The most frequent neurological complication of Waldenström's macroglobulinemia is IgM-mediated polyneuropathy. Direct tumor cell infiltration of the nervous system is very rare and better known as the “Bing and Neel syndrome”. This syndrome relates usually to a meningeal or meningo-myelo-cerebral tumor infiltration.

Observation

A 54-year-old man developed a terminal cauda equina syndrome over several years. MRI disclosed lumbar roots infiltration and lumbar puncture the presence of lymphocytic meningitis with intrathecal synthesis of monoclonal IgM identical to that found in the blood. The bone biopsy revealed a lymphoplasmocytic infiltration consistent with the diagnosis of Waldenström's macroglobulinemia. The final diagnosis was meningeal and lumbar roots infiltration revealing Waldenström's macroglobulinemia. Partial remission was obtained after polychemotherapy with CHOP, rituximab and methotrexate. At the end of the treatment, the patient improved his bladder's control and was able to walk with a stick.

Discussion

We reviewed the 35 cases of “Bing and Neel syndrome” we have identified by a PubMed research. The present case report is original by the initial neurological presentation of the disease three years before diagnosis and the successful use of rituximab in the polychemotherapy regimen.