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Bing-Neel Syndrome: A Rare Mimic of Secondary Normal Pressure Hydrocephalus
Neurology ( IF 9.9 ) Pub Date : 2021-11-30 , DOI: 10.1212/wnl.0000000000012917
Yosef Ellenbogen 1 , Afnan Al-Khotani 1 , David Tang-Wai 1 , Alfonso Fasano 1 , Ronit Agid 1 , Mojgan Hodaie 1
Affiliation  

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



中文翻译:

Bing-Neel 综合征:继发性常压脑积水的罕见模拟物

一名 80 岁男性,从 Waldenström 巨球蛋白血症 (WM) 中缓解,出现 6 个月的认知能力下降、广泛的拖曳步态和夜尿。系列 CT 头显示进行性脑室扩大。MRI 显示侧脑室液-液水平和外周脑沟/基底池异常的液体衰减倒置恢复信号。在额部硬脑膜、海绵窦和 Meckel 腔观察到硬脑膜增厚/增强(图)。脊柱 MRI 显示沿胸脊髓/圆锥增强,马尾神经增厚。高蛋白 CSF (7.01 g/L) 在腰椎穿刺后立即形成果冻状沉淀物(视频 1)。脑脊液白细胞计数为 27 x 10 6/L(92% 淋巴细胞)和流式细胞仪分析证实复发性 CNS WM(Bing-Neel 综合征)。1,2

更新日期:2021-11-29
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