April 2019
A 63-year-old man with IgM kappa restricted Waldenstrom macroglobulinemia (WM) presented with acute confusion.
Bing Neel Syndrome
- Background
- Bing-Neel syndrome (BNS) is a rare neurologic complication of Waldenström macroglobulinemia (WM) that was first described in 1936. It is associated with central nervous system infiltration by neoplastic lymphoplasmacytoid and plasma cells.
- Clinical Presentation
- Neurologic symptoms in patients with WM should raise suspicion of BNS. The most commonly reported symptoms of BNS are headache, ataxia, and progressive cognitive decline. Focal neurology of the tumoral subtype includes cranial nerve palsy.
- Key Diagnostic Features
- Blood tests usually show the typical features of WD. Diagnosis is made on cerebrospinal fluid testing and radiological imaging. MR demonstrates regions of enhancement within periventricular white matter with associated FLAIR hyperintensity. BNS can be subdivided into diffuse and tumoral forms. In the diffuse infiltrative form, the cerebral lymphoid infiltration predominates in the brainstem, periventricular white matter, and leptomeningeal spaces. In the tumoral form, the lesion may be unifocal or multifocal, and is usually located in the deep subcortical hemispheric regions.
- Differential Diagnosis
- GBM (a thick irregular ring of tissue around a necrotic core with marked contrast),
multifocal leukoencephalopathy (multifocal oval or round subcortical white matter hyperintensities in the parieto-occipital area), - Primary CNS lymphoma (PCL; PCLs in immunologically-normal patients usually enhance strongly and homogenously, while AIDS-related PCLs may appear considerably more heterogeneous).
- GBM (a thick irregular ring of tissue around a necrotic core with marked contrast),
- Treatment
- There is still no consensus on the treatment of BNS. Remission has been reported either with intrathecal and/or systemic chemotherapies, including high-dose methotrexate or cytarabine and Ibrutinib, and with whole brain radiation therapy, alone or in combination with chemotherapy.
