Bing-Neel Syndrome: A Rare Mimic of Secondary Normal Pressure Hydrocephalus

An 80-year-old man, in remission from Waldenström macroglobulinemia (WM), presented with 6-month cognitive decline, broad-based shuffling gait, and nocturia. Serial CT head demonstrated progressive ventriculomegaly. MRI revealed abnormal fluid-attenuated inversion recovery signal in the lateral ventricles with fluid–fluid levels and in peripheral sulci/basal cisterns. Pachymeningeal thickening/enhancement was noted at the frontal dura, cavernous sinus, and Meckel caves (Figure). Spinal MRI revealed enhancement along the thoracic spinal cord/conus with a thickened cauda equina. Highly proteinaceous CSF (7.01 g/L) formed a jelly-like precipitate moments after lumbar puncture (Video 1). CSF white blood cell count was 27 x 106/L (92% lymphocyte) and flow cytometric analysis confirmed recurrent CNS WM (Bing-Neel syndrome).1,2

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