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

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. 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. Seventeen studies encompassing 456 patients met the inclusion criteria. The pooled recurrence rate was 23% (95% CI, 17–32%), with substantial heterogeneity (I2 = 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. 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.

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Journal
Neurological Sciences
Published
2026-03-26
DOI
https://doi.org/10.1007/s10072-026-08985-7
Primary Topic
IgG4-Related and Inflammatory Diseases
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article
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article

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

Bernardo Leite Pondé da Luz, Guilherme Diogo Silva
Neurological Sciences
IgG4-Related and Inflammatory Diseases
article

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

Bernardo Leite Pondé da Luz, Guilherme Diogo Silva
article en

Abstract

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. 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. Seventeen studies encompassing 456 patients met the inclusion criteria. The pooled recurrence rate was 23% (95% CI, 17–32%), with substantial heterogeneity (I2 = 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. 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.

Neurological SciencesVol. 47(4)
Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (BR)
Reduced inequalities
Openalex Percentile: Top 6%
IgG4-Related and Inflammatory Diseases
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Relapse in Tolosa-Hunt syndrome: pooled recurrence rates and associated factors from a meta-analysis of 456 cases — Bernardo Leite Pondé da Luz, Guilherme Diogo Silva · Neurological Sciences (2026) | TGRS Research Map | TGRS