痛性眼肌麻痹综合征的临床观察

Clinical observation on painful ophthalmoplegia syndrome

目的观察痛性眼肌麻痹综合征(THS)的临床表现及预后特点,并探讨其发病原因。方法回顾性病例研究。临床检查确诊首发痛性眼肌麻痹综合征的患者31例纳入本研究。其中男性19例,女性12例,年龄26~87岁,平均(57.42±13.99)岁;所有患者入院后均行头颅MRI、血常规、血红蛋白、生化全套、尿常规及自身抗体等检查,必要时行腰椎穿刺。确诊后给予糖皮质激素冲击和或免疫抑制剂治疗。随访1年,观察患者的预后特点,并分析其发病的原因。结果 31例患者起病不同,发现3例患有系统性红斑狼疮,2例患有干燥综合征。主要临床表现差别不大,均首先出现头痛,随后出现同侧眼眶痛、眼睑下垂、眼球运动受限等,支配眼球的颅神经以动眼神经受累最多见,占77.4%,其次为外展神经,占19.4%;滑车神经受累较少见,占3.2%。入院治疗后,31例患者头痛均于7 d内完全缓解,平均时间(3.64±1.58)d;29例患者眼球症状于8周内完全缓解,平均时间(4.90±2.18)周;2例患者遗留痛性眼肌麻痹后遗症。完全恢复未复发者共26例,占83.9%;完全恢复又复发者共3例,占9.7%;未完全恢复者共2例,占6.5%。结论痛性眼肌麻痹综合征发病原因不明,可能与自身免疫性疾病相关,主要临床表现相似,糖皮质激素冲击和或免疫抑制剂治疗有效,但可能复发,临床需定期随访。

Objective To observe the clinical manifestation and prognosis of painful ophthalmoplegia syndrome（ Tolosa-Hunt syndrome）,and to explore its pathogenesis. Methods A retrospective case study. 31 patients who were diagnosed with painful ophthalmoplegia syndrome were recruited for the study. Among these 31 subjects,there were 19 male and 12 female,had an average age of 57. 42 ± 13. 99 years old（ ranging from 26 to 87）. All the patients underwent head MRI,hemoglobin,blood routine,urine routine,biochemical test,autoantibody test and so on,and given glucocorticoid and/or immunosuppressant treatment after diagnosis. Follow up 1 year,observe the clinical manifestation and prognosis of patients with the disease,and analyze the pathogenesis. Results 31 patients with different onset,discover 3 patients with systemic lupus erythematosus and 2 patients with Sjogren＇s syndrome. All patients clinical manifestations are similar,the first headache,followed by ipsilateral orbital pain,ptosis,eye movement restriction and so on. The cranial nerve of the eyeballs is the most common for the oculomotor nerve involvement,accounting for 77. 4%,followed by abduction nerve,accounting for 19. 4%,and the trochlear nerve involvement is rare,accounting for 3. 2%. After the treatment,all patients with headache complete remission within 7 days,the average time（ 3. 64 ＋ 1. 58） days; 29 cases of complete remission in patients with ocular symptoms within 8 weeks,the average time（ 4. 90 ＋ 2. 18） weeks; 2 cases had sequela of the painful ophthalmoplegia syndrome. 26 patients complete recovery without recurrence,accounting for 83. 9%; 3 patients complete recovery but recurrence,accounting for 9. 7%; 2 patients incomplete recovery,accounting for 6. 5%. Conclusions The pathogenesis of the painful ophthalmoplegia syndrome is unknown. It may be associated with autoimmune diseases. The main clinical manifestations are similar,glucocorticoid and/or immunosuppressant treatment is effective,but may recurrence,so need clinical follow-up regularly.