Clinical profile and outcomes in Tolosa-Hunt Syndrome; a systematic review

Introduction

Tolosa-Hunt Syndrome (THS) stands as a rare headache disorder distinguished by painful ophthalmoplegia, accompanied by headaches and cranial nerve palsies. The syndrome was initially identified by Eduardo Tolosa in Spain in 1954. He observed granulomatous inflammation surrounding a carotid siphon in a patient with an intracavernous carotid aneurysm. The objective of this systematic review is to consolidate and summarize existing studies on THS, providing a comprehensive evaluation of its clinical findings and outcomes.

Methods

This review adhered to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) checklist. Systematic searches were conducted on PubMed and Scopus databases to identify literature examining the sociodemographics, clinical findings, cranial nerve palsies, laboratory and radiological data, treatment, and outcomes of THS. The study followed a pre-established protocol registered on the PROSPERO database (ID: CRD42023494249).

Results

Out of 1115 studies screened, 11 met the predefined inclusion and exclusion criteria. The studies predominantly focused on Asian populations, emphasizing unilateral orbital headaches as a common clinical feature. Ophthalmological findings, including restriction of eye movements, diplopia, ptosis, and vision loss, were prevalent. Studies also highlighted some cases presenting atypically without ophthalmoplegia but with acute vision changes. Oculomotor nerve palsy, followed by abducens and trochlear nerve palsies, were the most frequently reported. Laboratory investigations across the studies often showed normal cerebrospinal fluid findings and varying levels of inflammatory markers like ESR and CRP. Inflammation of the cavernous sinus and orbital apex was noted most frequently. Treatment strategies were consistent across the studies, with steroids (both IV and oral) being the mainstay treatment for managing THS. Despite the use of steroids, the studies reported varied outcomes in terms of pain relief and recovery from cranial nerve deficits, with some cases showing rapid improvement while others had prolonged or incomplete recovery. Other immunosuppressants and steroid sparing agents are used with varying levels of success. Recurrence rates ranged from 9% to 71% across studies.

Conclusion

This review discusses the varied constellation of symptoms associated with THS, with headaches and cranial nerve findings being consistently observed. High and low doses, as well as both intravenous and oral steroids, have proven to be effective in managing THS. Overall, the prognosis appears favorable, with a limited number of cases showing recurrence.