Kim Ho-Jung, Suh Sang-Il, Kim Joo Han, Kim Byung-Jo
Department of Neurology, Korea University College of Medicine, Seoul, Korea.
J Korean Neurosurg Soc. 2009 Dec;46(6):588-91. doi: 10.3340/jkns.2009.46.6.588. Epub 2009 Dec 31.
Radiologic findings of Bing-Neel syndrome, which is an extremely uncommon complication resulting from malignant lymphocyte infiltration into the central nervous system (CNS) in patients with Waldenström's macroglobulinemia (WM), have been infrequently reported due to extreme rarity of the case. A 75-year-old man with WM presented at a neurology clinic with progressive gait and memory disturbances, and dysarthria of 2 months duration. Cerebrospinal fluid and serum protein electrophoresis and immunofixation electrophoresis showed IgM kappa-type monoclonal gammopathy. Brain magnetic resonance imaging revealed multifocal, hyperintense lesions on T2 weighted-images. Brain diffusion-weighted imaging (DWI) demonstrated hyperintensities in cerebral and cerebellar lesions that appeared isointense on apparent diffusion coefficient maps, which were compatible with vasogenic edema. Although histologic analysis is a confirmative study to prove direct cell infiltration into the brain, brain MRI with DWI may be a good supportive study to diagnose Bing-Neel syndrome.
宾-尼尔综合征是华氏巨球蛋白血症(WM)患者中恶性淋巴细胞浸润中枢神经系统(CNS)导致的一种极为罕见的并发症,由于病例极其罕见,其放射学表现鲜有报道。一名75岁的WM男性患者因进行性步态和记忆障碍以及持续2个月的构音障碍就诊于神经科门诊。脑脊液和血清蛋白电泳及免疫固定电泳显示IgM κ型单克隆丙种球蛋白病。脑磁共振成像在T2加权像上显示多灶性高信号病变。脑弥散加权成像(DWI)显示大脑和小脑病变呈高信号,在表观扩散系数图上呈等信号,符合血管源性水肿。尽管组织学分析是证实细胞直接浸润脑内的确定性研究,但联合DWI的脑MRI可能是诊断宾-尼尔综合征的良好辅助检查。