Ata Fateen, Yousaf Zohaib, Arachchige Suresh Nalaka Menik, Rose Saman, Alshurafa Awni, Muthanna Bassam, Bilal Ammara Bint I, El Beltagi Ahmed, Zahid Muhammad
Department of Internal Medicine, Hamad General Hospital, Hamad Medical Corporation, Doha, Qatar.
Department of Radiology, Hamad General Hospital, Hamad Medical Corporation, Doha, Qatar.
eNeurologicalSci. 2021 Jul 22;24:100359. doi: 10.1016/j.ensci.2021.100359. eCollection 2021 Sep.
Tolosa Hunt syndrome (THS) is a rare disease that manifests mainly as painful unilateral ophthalmoplegia. It is caused by an inflammatory process of unknown aetiology within the cavernous sinus with a rare intracranial extension. The International Classification of Headache Disorders (ICHD)- 3 diagnostic criteria aids in its diagnosis. There is limited literature on its varied presentations, diagnosis, and management. Steroids are used in the treatment of THS with varied success.
We conducted a single-center-retrospective-study and included all patients admitted with a diagnosis of THS from January 2015 to December 2020. Descriptive and summary statistics were used to describe the study cohort's socio-demographic parameters.
Among 31 THS patients (predominantly Asians (18) and Arabs (9)), visual disturbance was commonest presenting complaint. Third-nerve paralysis was seen in 70.9% cases. Magnetic-resonance-imaging (MRI) was abnormal in 64.5%. 93.5% patients received steroids, with a response-rate of 70.9% and a recurrence-rate of 9.7%. A previous history of THS and female gender were associated with recurrence (-value 0.009 and 0.018). Recurrence was seen in 66.7% fully recovered and 33.3% partially recovered cases (p-value 0.04). Among the benign and inflammatory subtypes of THS, the ICHD-3 criteria were applicable in 85% of inflammatory THS.
THS is a rare disease with ethnic variation in presentation and response to treatment. In our cohort female gender and a previous history of THS were associated with recurrence. ICHD-3 diagnostic criteria had a higher validity in our patients compared to prior studies, especially among the inflammatory THS.
托洛萨-亨特综合征(THS)是一种罕见疾病,主要表现为单侧疼痛性眼肌麻痹。它由海绵窦内病因不明的炎症过程引起,颅内扩展罕见。《国际头痛疾病分类》(ICHD)-3诊断标准有助于其诊断。关于其多样表现、诊断和治疗的文献有限。类固醇用于治疗THS,疗效各异。
我们进行了一项单中心回顾性研究,纳入了2015年1月至2020年12月期间所有诊断为THS的住院患者。采用描述性和汇总统计来描述研究队列的社会人口统计学参数。
在31例THS患者中(主要是亚洲人(18例)和阿拉伯人(9例)),视力障碍是最常见的主诉。70.9%的病例出现动眼神经麻痹。64.5%的患者磁共振成像(MRI)异常。93.5%的患者接受了类固醇治疗,有效率为70.9%,复发率为9.7%。既往有THS病史和女性与复发相关(P值分别为0.009和0.018)。66.7%完全康复和33.3%部分康复的病例出现复发(P值为0.04)。在THS的良性和炎症亚型中,ICHD-3标准适用于85%的炎症性THS。
THS是一种罕见疾病,在表现和对治疗的反应上存在种族差异。在我们的队列中,女性和既往有THS病史与复发相关。与先前研究相比,ICHD-3诊断标准在我们的患者中具有更高的有效性,尤其是在炎症性THS中。