Capasso Raffaella, Buonincontro Miriam, Caranci Ferdinando, Pinto Antonio
Department of Radiology, CTO Hospital, Azienda Ospedaliera dei Colli, Viale Colli Aminei 21, 80141 Naples, Italy.
Department of Pulmonology and Oncology, Respiratory Intensive Care Unit, Monaldi Hospital, 80131 Naples, Italy.
Reports (MDPI). 2024 May 7;7(2):34. doi: 10.3390/reports7020034.
Bing-Neel syndrome (BNS) is a rare condition that may occur in patients with Waldenstrom macroglobulinemia (WM) and is caused by lymphoplasmacytic infiltration into the central nervous system. BNS is an extramedullary manifestation of WM which may present with various neurological signs and symptoms that make the diagnosis difficult to achieve. We present a case of BNS in a 60-year-old patient diagnosed 6 years after recovering from Waldenstrom's macroglobulinemia. We observed the patient for a secondary generalized focal motor seizure. Unenhanced brain CT revealed slight hyperdensity of left parietal subarachnoid spaces. The MRI of the brain and spinal cord showed leptomeningeal enhancement in both parietal lobes. The presence of monoclonal bands (light chain k and IgM) was found in cerebrospinal fluid, leading to the diagnosis of BNS. The patient started treatment with ibrutinib and remains clinically stable during a 1-year follow-up. However, the MRI showed the appearance of a new subcortical left parietal lesion. BNS is an extremely rare presentation of WM that should be recognized and considered early in the presence of unexplained neurological symptoms in patients with a history of WM, even if the patient appears to have recovered.
宾-尼尔综合征（BNS）是一种罕见疾病，可能发生在华氏巨球蛋白血症（WM）患者中，由淋巴浆细胞浸润中枢神经系统所致。BNS是WM的一种髓外表现，可出现各种神经体征和症状，导致诊断困难。我们报告一例60岁患者，在从华氏巨球蛋白血症康复6年后被诊断为BNS。我们观察该患者出现继发性全身性局灶性运动性癫痫发作。未增强的脑部CT显示左侧顶叶蛛网膜下腔轻度高密度。脑部和脊髓的MRI显示双侧顶叶软脑膜强化。脑脊液中发现单克隆条带（轻链κ和IgM），从而诊断为BNS。患者开始使用伊布替尼治疗，在1年的随访期间临床保持稳定。然而，MRI显示左侧顶叶皮质下出现新病变。BNS是WM极其罕见的表现，对于有WM病史且出现无法解释的神经症状的患者，即使患者似乎已康复，也应尽早认识并考虑到该病。
