华氏巨球蛋白血症 (WM) 是一种低级别 B 细胞肿瘤。 Bing Neel 综合征是 WM 的一种罕见表现，其特征是中枢神经系统浸润性受累。64 岁男性，有 4 年缓慢进行性复视和眼睑下垂病史。检查显示左侧动眼神经（内眼肌和外眼肌麻痹），滑车神经、外展神经以及右侧部分动眼神经和外展神经受累。评估显示血红蛋白贫血 10.7 g/dL，红细胞沉降率升高 120 mm/h，血浆白蛋白：球蛋白逆转。血清蛋白电泳显示早期 γ 区有副蛋白峰，IgM 水平升高 (3810 mg/dL)，游离 kappa 轻链水平升高 (70.1 mg/L)。髂后嵴骨髓抽吸显示成熟小淋巴细胞，免疫组织化学标记物 CD5 阳性，CD10 阴性，MYD88 突变阳性，提示 WM。患者接受苯达莫司汀和利妥昔单抗方案治疗，一年后神经功能没有改善。该病例扩大了副蛋白血症性神经病的范围，包括颅神经麻痹。因此，对于孤立性眼肌轻瘫患者，尤其是老年患者，即使患有糖尿病等其他合并症，也必须考虑浆细胞恶液质。© 2024 作者。

Waldenstroms macroglobulinemia (WM) is a low-grade B cell neoplasm. Bing Neel syndrome is a rare manifestation of WM characterized by infiltrative involvement of the central nervous system.64-year-old man, presented with 4 years history of slowly progressive diplopia and ptosis of eyes. Examination showed left oculomotor (internal and external ophthalmoplegia), with trochlear, abducens, and right partial oculomotor and abducens nerve involvement. Evaluation showed anemia of hemoglobin 10.7 g/dL, raised erythrocyte sedimentation rate of 120 mm/h and plasma albumin:globulin reversal. Serum protein electrophoresis showed a paraprotein peak in the early gamma region with elevated IgM level (3810 mg/dL) and elevated free kappa light chain level (70.1 mg/L). Bone marrow aspiration from posterior iliac crest revealed mature small lymphocytes with positive immunohistochemical markers of CD5, CD10 negativity and MYD88 mutation positivity suggestive of WM. Patient was treated with bendamustine and rituximab regimen, with no neurological improvement at the end of one year.This case expands spectrum of paraproteinemic neuropathy to include cranial nerve palsy. Thus, plasma cell dyscrasias have to be considered in patients with isolated ophthalmoparesis especially in elderly patients, even with other comorbidities such as diabetes mellitus.© 2024 The Authors.