Relapse in Tolosa-Hunt syndrome: pooled recurrence rates and associated factors from a meta-analysis of 456 cases

Tolosa-Hunt综合征复发:456例病例荟萃分析的汇总复发率及相关因素

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Abstract

OBJECTIVE: Tolosa-Hunt Syndrome (THS) is a rare, painful ophthalmoplegia caused by granulomatous inflammation within the cavernous sinus. Despite the effective response to corticosteroid therapy, recurrence rates reported in the literature vary significantly, and clear associated factors of recurrence remain elusive. This systematic review and meta-analysis aim to establish the frequency of relapse and identify f actors associated with recurrence. EVIDENCE ACQUISITION: Following PRISMA guidelines, we performed a comprehensive database search of MEDLINE/PubMed, EMBASE, and Scopus from inception to June 2024, focusing on observational studies or case series reporting THS recurrence. Meta-analysis employed a random-effects model to estimate pooled recurrence rates. Meta-regression analyses evaluated clinical and demographic associated factors of recurrence, with publication bias assessed via funnel plots and Egger’s test. RESULTS: Seventeen studies encompassing 456 patients met the inclusion criteria. The pooled recurrence rate was 23% (95% CI, 17–32%), with substantial heterogeneity (I(2) = 59.9%). The frequency of cranial nerve involvement specifically nerves II, IV, and VI, correlated with higher recurrence risk (P < 0.05). Age, sex, duration of follow-up, and MRI abnormalities did not show significant associations. Limited data were available concerning the use of steroid-sparing immunosuppressants, inflammatory biomarkers, and characteristics of MRI. CONCLUSION: Approximately one-quarter of patients with THS experience recurrence. Involvement of cranial nerves II, IV, and VI was associated with relapses. Further prospective investigations with standardized recurrence definitions and structured therapeutic comparisons are needed to clarify prognostic factors and optimize long-term management. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s10072-026-08985-7.

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