Objective: To investigate the clinical features of Tolosa-Hunt syndrome (THS), a type of painful ophthalmoplegia. Methods: This was a retrospective case series study. The clinical data of patients diagnosed with painful ophthalmoplegia in the Department of Neurology of Tianjin Medical University General Hospital from January 2019 to December 2022 were continuously collected. THS and other types of painful ophthalmoplegia were diagnosed according to the criteria of the International Classification of Headache Disorders, 3rd Edition. The first symptoms, ocular manifestations, imaging and laboratory findings, treatment (glucocorticoids in THS patients) and outcomes were recorded and compared between patients with THS and the other patients. Results: A total of 35 patients with painful ophthalmoplegia who met the criteria were included. Eighteen patients were diagnosed with THS, including 11 males and 7 females, with an average age of (59.9±13.2) years old (range, 28 to 80 years old). Seventeen patients presented with pain as the initial symptom, 17 patients had ptosis, and 10 patients had abnormal signal lesions in the orbital and cavernous sinus area on MRI on the affected side. Nine patients had abnormal serum immunological results (3 had elevated erythrocyte sedimentation rate and C-reactive protein levels, 6 had positive antinuclear antibodies, and 1 was diagnosed with systemic lupus erythematosus and rheumatoid arthritis). Among the 17 patients with other types of painful ophthalmoplegia, 12 had concurrent diabetes mellitus (including 1 with positive syphilis antibodies and 1 with active pulmonary tuberculosis), 2 had intracranial aneurysms, 1 had ischemic oculomotor nerve palsy, 1 had intracranial infection, and 1 had basal meningitis. Five THS patients experienced symptom relief after 3 days of corticosteroid treatment, and 13 THS patients experienced relief within 1 to 6 weeks of treatment. During the follow-up period (7 to 47 months), 3 patients with THS relapsed. Conclusions: Most patients with THS have pain as the first symptom, with positive signs of cavernous sinus on MRI and abnormal serum immunologic examination results. Other types of painful ophthalmoplegia usually combine with diabetes mellitus or other systemic diseases, which is helpful in the differential diagnosis.
目的： 探讨Tolosa-Hunt综合征（THS）痛性眼肌麻痹的临床特征。 方法： 回顾性病例系列研究。连续收集2019年1月至2022年12月在天津医科大学总医院神经科诊断为痛性眼肌麻痹患者的临床资料，根据《国际头痛疾病分类》第3版标准确诊THS患者和其他类型痛性眼肌麻痹患者。记录和比较两类患者的首发症状、眼部表现、影像学和实验室检查结果、治疗（THS患者接受糖皮质激素治疗）及随访情况。 结果： 共收集35例符合标准的痛性眼肌麻痹患者临床资料。18例患者诊断为THS，男性11例，女性7例；年龄为（59.9±13.2）岁，年龄范围为28~80岁；其中17例患者以疼痛为首发症状，17例患者合并上睑下垂，10例患者受累侧MRI眼眶及海绵窦区域异常信号病灶；9例患者合并血清免疫学检查结果异常（3例红细胞沉降率和C反应蛋白水平升高，6例抗核抗体阳性，1例确诊为系统性红斑狼疮及类风湿性关节炎）。在17例其他类型痛性眼肌麻痹患者中，合并糖尿病12例（包含梅毒抗体阳性1例、活动性肺结核1例），颅内动脉瘤2例，缺血性动眼神经麻痹、颅内感染、颅底脑膜炎各1例。5例THS患者糖皮质激素治疗3 d症状缓解，13例治疗1~6周症状缓解。随访7~47个月，3例THS患者复发。 结论： 多数THS痛性眼肌麻痹患者以疼痛为首发症状，MRI检查可见海绵窦阳性体征，并合并血清免疫学检查结果异常；其他类型痛性眼肌麻痹患者合并糖尿病及其他系统性疾病，有助于鉴别诊断。.