Ueki Syugo, Nakamura Masataka, Sasaki Risa, Okada Yoichiro, Yoshikawa Keisuke, Kusunoki Susumu, Ishii Kazuyoshi, Kusaka Hirofumi, Kondo Takayuki
Department of Neurology, Kansai Medical University Medical Center, Moriguchi, Japan.
Department of Neurology, Kansai Medical University, Hirakata, Japan.
Case Rep Neurol. 2018 Mar 28;10(1):88-94. doi: 10.1159/000487850. eCollection 2018 Jan-Apr.
A 71-year-old man with Waldenström macroglobulinemia (WM) presented with a slowly progressive sensory disturbance and mild weakness predominantly affecting the distal portion of the limbs over the course of 6 months. Cervical magnetic resonance imaging (MRI) showed a long hyperintense lesion at the C1-C4 level. Nerve conduction studies (NCS) revealed prolongation of distal latency, slowed conduction velocity, and conduction block. His serum IgM level was increased, and he was positive for anti-myelin-associated glycoprotein (MAG) and anti-sulfoglucuronyl paragloboside (SGPG) IgM antibodies. Based on the presence of anti-MAG/SGPG antibodies and a single atypical cell with lymphoplasmacytic character in the cerebral spinal fluid, he was diagnosed as having anti-MAG/SGPG neuropathy and Bing-Neel syndrome (BNS) associated with WM. Following 6 cycles of bendamustine monotherapy, the patient's neurological impairment improved; and the serum IgM level became normalized. Furthermore, NCS findings indicated improvement; and the hyperintense lesion on MRI had almost completely disappeared. The present findings suggest that bendamustine monotherapy is effective not only for WM but also for its associated MAG/SGPG neuropathy and BNS.
一名71岁的患有华氏巨球蛋白血症（WM）的男性患者，在6个月的病程中出现了缓慢进展的感觉障碍和轻度无力，主要影响四肢远端。颈椎磁共振成像（MRI）显示C1 - C4水平有一个长T2高信号病变。神经传导研究（NCS）显示远端潜伏期延长、传导速度减慢和传导阻滞。他的血清IgM水平升高，抗髓鞘相关糖蛋白（MAG）和抗硫酸葡萄糖醛酸副球蛋白（SGPG）IgM抗体呈阳性。基于脑脊液中抗MAG/SGPG抗体的存在以及一个具有淋巴浆细胞特征的单个非典型细胞，他被诊断为患有抗MAG/SGPG神经病和与WM相关的宾-尼尔综合征（BNS）。在接受6个周期的苯达莫司汀单药治疗后，患者的神经功能障碍有所改善；血清IgM水平恢复正常。此外，NCS结果显示有所改善；MRI上的高信号病变几乎完全消失。目前的研究结果表明，苯达莫司汀单药治疗不仅对WM有效，而且对其相关的MAG/SGPG神经病和BNS也有效。
