The demographics of Tolosa-Hunt syndrome in Qatar

Abstract

Background: 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. Methods: 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. Results: 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 (p-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. Conclusions: 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.

Keywords

• Tolosa Hunt syndrome
• THS
• Cavernous sinus
• Ophthalmoplegia
• Third nerve palsy