一位55岁女性患者出现新发头痛、头皮疼痛、肩关节痛、盗汗和食欲不振症状。她被初级医生诊断为巨细胞动脉炎，并开始口服皮质类固醇治疗。然而，她的头痛、头皮疼痛和盗汗症状持续存在。随后她出现右侧霍纳综合征和三叉神经感觉减退。详细的血液检查显示炎症标志物轻度升高和寡克隆蛋白血症。随后骨髓活检显示淋巴浆细胞混合型淋巴瘤，造血功能占10%，分期诊断为无淋巴结或中枢神经系统损害的Waldenstrom巨球蛋白血症。颞动脉活检的免疫组化染色显示血管周围淋巴浆细胞细胞和寡克隆蛋白沉积。她被诊断为巨球蛋白血症-Bing-Neel综合征（BNS）的中枢神经系统受累。对于已经接受激素免疫抑制治疗的患者，鉴别淋巴瘤罕见的中枢神经系统受累是具有挑战性的。在缺乏明确诊断标准的情况下，这种罕见且多样化的BNS仍然是一种临床诊断。 版权所有©2023年北美神经-眼科学会。

A 55-year-old woman presented with new-onset headache, scalp tenderness, shoulder arthralgias, night sweats, and loss of appetite. She was diagnosed with giant cell arteritis by her primary care physician and commenced on oral corticosteroids. However, her headache, scalp tenderness, and night sweats persisted. She then developed right Horner syndrome and trigeminal hypoesthesia. Extensive blood work-up revealed mildly elevated inflammatory markers and a paraproteinemia. Subsequent bone marrow biopsy showed lymphoplasmacytic lymphoma, with 10% of hemopoiesis, and staging led to the diagnosis of Waldenstrom macroglobulinemia without nodal or central nervous system (CNS) lesions. Immunohistochemical staining of a temporal artery biopsy showed perivascular lymphoplasmacytic cells and paraprotein deposits. She was diagnosed with CNS involvement of her macroglobulinemia-Bing-Neel syndrome (BNS). Identification of rare CNS involvement of lymphoma is challenging when a patient is already on steroid immunosuppression. In the absence of clear diagnostic criteria, the rare and heterogenous BNS remains a clinical diagnosis.Copyright © 2023 by North American Neuro-Ophthalmology Society.