Kahl Nicolas, Pelucio Maria
University of California, San Diego, Department of Emergency Medicine, San Diego, CA.
J Educ Teach Emerg Med. 2022 Jan 15;7(1):V22-V25. doi: 10.21980/J8KW7G. eCollection 2022 Jan.
A 39-year-old previously healthy male presented with three days of right eye pressure and one day of binocular diplopia. He denied history of trauma, headache, or other neurological complaints. He had normal visual acuity, normal intraocular pressure, intact convergence, and no afferent pupillary defect. His neurologic examination was non-focal except for an inability to adduct the right eye past midline on cranial nerve examination, with left-beating nystagmus noted in the left eye. MRI brain was obtained, showing right cavernous sinus lateral wall contrast enhancement. No other evidence of neoplasm, ischemia, infection, or aneurysm was found. The patient's deficits rapidly improved with corticosteroids. This is the hallmark of Tolosa-Hunt syndrome (THS), characterized as idiopathic inflammation of the cavernous sinus. The patient was discharged on a prednisone taper, with continued improvement at outpatient neurology follow-up. Tolosa-Hunt syndrome is one of the rare disorders recognized by the National Organization for Rare Disorders (NORD), and has an estimated incidence of one case per million per year.
Tolosa-Hunt syndrome, cranial nerve deficits, diplopia, neurologic examination.
一名39岁既往健康男性，出现右眼胀痛3天及双眼复视1天。他否认有外伤、头痛或其他神经系统症状史。其视力正常、眼压正常、集合功能正常，且无传入性瞳孔障碍。除了在颅神经检查时右眼不能内收超过中线，且左眼可见向左跳动的眼球震颤外，其神经系统检查无局灶性异常。行头颅MRI检查，显示右侧海绵窦外侧壁有对比增强。未发现肿瘤、缺血、感染或动脉瘤的其他证据。患者的症状在使用糖皮质激素后迅速改善。这是托洛萨-亨特综合征（THS）的特征，其特征为海绵窦的特发性炎症。患者出院时逐渐减量使用泼尼松，在门诊神经科随访时持续好转。托洛萨-亨特综合征是国家罕见病组织（NORD）认可的罕见疾病之一，估计每年发病率为百万分之一。
托洛萨-亨特综合征、颅神经缺损、复视、神经系统检查。
