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以持续性单侧头痛和疼痛性眼肌麻痹为表现的托洛萨-亨特综合征:一例罕见病例报告,凸显诊断挑战及治疗反应

Tolosa-Hunt syndrome presenting as persistent unilateral headache and painful ophthalmoplegia: A rare case report highlighting diagnostic challenges and therapeutic response.

作者信息

Yadav Riya, Parihar Pratapsingh, Bhangale Paritosh, Jajoo Umang

机构信息

Department of Radiodiagnosis, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education & Research, Wardha, India.

出版信息

Radiol Case Rep. 2025 Apr 17;20(7):3340-3343. doi: 10.1016/j.radcr.2025.03.084. eCollection 2025 Jul.

Abstract

Tolosa-Hunt syndrome (THS) is a rare idiopathic granulomatous inflammation of the cavernous sinus, superior orbital fissure, or orbital apex, presenting with painful ophthalmoplegia. The exact etiology remains unclear, but it is believed to be immune-mediated. Early diagnosis is essential, as prompt corticosteroid therapy results in rapid symptom resolution. This case report highlights the clinical presentation, diagnostic workup, and management of a patient with Tolosa-Hunt syndrome, emphasizing the role of neuroimaging in confirming the diagnosis and the efficacy of corticosteroid treatment. A 55-year-old male presented with a persistent right-sided headache for 2.5 months, accompanied by diplopia for 5 days. Neurological and ophthalmic examination revealed right-sided ptosis, restricted extraocular movements, and binocular diplopia. MRI findings demonstrated an asymmetric enlargement of the right cavernous sinus with inflammatory changes extending into the orbital apex and superior orbital fissure, confirming Tolosa-Hunt syndrome. The patient was treated with high-dose corticosteroids, leading to significant clinical improvement. Following corticosteroid therapy, the patient experienced rapid resolution of headache and improvement in ocular motility. Repeat MRI after 6 weeks showed a marked reduction in inflammation. There was no recurrence of symptoms upon gradual steroid tapering. Tolosa-Hunt syndrome should be considered in patients with unilateral headache and painful ophthalmoplegia. MRI plays a crucial role in diagnosis, and corticosteroids remain the mainstay of treatment, leading to excellent outcomes. Early recognition and intervention can prevent long-term complications and disability.

摘要

托洛萨-亨特综合征(THS)是一种罕见的海绵窦、眶上裂或眶尖特发性肉芽肿性炎症,表现为疼痛性眼肌麻痹。确切病因尚不清楚,但据信是免疫介导的。早期诊断至关重要,因为及时使用皮质类固醇治疗可使症状迅速缓解。本病例报告强调了一名托洛萨-亨特综合征患者的临床表现、诊断检查和治疗,强调了神经影像学在确诊中的作用以及皮质类固醇治疗的疗效。一名55岁男性持续右侧头痛2.5个月,伴有复视5天。神经和眼科检查发现右侧上睑下垂、眼球运动受限和双眼复视。MRI检查结果显示右侧海绵窦不对称扩大,炎症改变延伸至眶尖和眶上裂,确诊为托洛萨-亨特综合征。该患者接受了高剂量皮质类固醇治疗,临床症状明显改善。皮质类固醇治疗后,患者头痛迅速缓解,眼球运动改善。6周后复查MRI显示炎症明显减轻。逐渐减少类固醇剂量后症状未复发。单侧头痛伴疼痛性眼肌麻痹的患者应考虑托洛萨-亨特综合征。MRI在诊断中起关键作用,皮质类固醇仍然是主要治疗方法,可取得良好效果。早期识别和干预可预防长期并发症和残疾。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9815/12035724/1be185abe6bd/gr1.jpg

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