Bing-Neel Syndrome

Bing-Neel syndrome refers to Waldenström macroglobulinemia with central nervous system infiltration. Waldenström macroglobulinemia is defined by the World Health Organization as a "lymphoplasmacytic lymphoma with bone marrow involvement and IgM monoclonal gammopathy of any concentration." Patients with Waldenström macroglobulinemia have anemia and lymphoplasmacytic infiltration of the bone marrow. Lymph nodes and the spleen are often also involved, but involvement of the central nervous system (Bing-Neel syndrome) is rare.

While patients with Waldenström macroglobulinemia can present with stroke and other focal or multifocal brain syndromes, diffuse encephalopathy, and/or subarachnoid hemorrhage due to hyperviscosity of blood or thrombocytopenia, Bing-Neel syndrome refers to perivascular infiltration of small lymphocytes, lymphoplasmacytoid cells, and plasma cells in the brain parenchyma and/or spine. The infiltration can be diffuse or tumoral. In diffuse Bing-Neel syndrome, lymphoid infiltration is seen predominantly in the pons, medulla, periventricular white matter, and leptomeningeal spaces. In the tumoral form, unifocal or multifocal lesions are most commonly located in the deep subcortical white matter.

Patients with Bing-Neel syndrome can present with seizures, confusion, cognitive decline, headache, blurry or cloudy vision, psychiatric symptoms, pain, numbness, paresthesias, hearing loss, and weakness.

On MRI, one may see enhancing lesions and/or thickening or enhancement of meningeal sheaths. Multiple infarctions can also be seen as a result of neoplastic vascular obstruction. The images above reveal pachymeningeal enhancement and FLAIR-hyperintensity.

References

• Kim HJ, Suh SI, Kim JH, Kim BJ. Brain magnetic resolution imaging to diagnose bing-neel syndrome. J Korean Neurosurg Soc. 2009 Dec;46(6):588-91.
• Malkani RG, Tallman M, Gottardi-Littell N, Karpus W, Marszalek L, Variakojis D, Kaden B, Walker M, Levy RM, Raizer JJ. Bing-Neel syndrome: an illustrative case and a comprehensive review of the published literature. J Neurooncol. 2010 Feb;96(3):301-12.