The clinical features and outcomes of Tolosa-Hunt syndrome

Shin Yeop Oh,Hyuna Kim

doi:10.1186/s12886-021-02007-0

Abstract

BackgroundThe objective of this study was to investigate the clinical features and outcomes of Tolosa-Hunt syndrome (THS).MethodsA retrospective review of the medical records was performed on patients with THS between March 2016 and January 2020. A total of eleven patients fulfilling the International Classification of Headache Disorders (ICHD-3 beta) diagnostic criteria for THS were included in this study.ResultsThe average age of the patients with THS was 57.18 ± 15.32 years and the mean duration of recovery was 26.91 ± 24.35 days. All eleven patients had orbital or periorbital pain as the first symptom followed by diplopia. Ptosis was found in five patients (45.45 %) in the involved eye. Sixth cranial nerve (CN) palsy was most common (eight cases, 72.73 %), followed by third and fourth CN palsy (five cases, 45.45 %, respectively), optic neuritis (two cases, 18.18 %), and trigeminal nerve and facial nerve palsy (one case, 9.09 %, respectively). One patient with optic neuritis failed to recover visual acuity and the other ten patients completely recovered their ocular motor limitation. All patients were initially treated with steroids. One patient relapsed after five weeks and one patient had a history of THS five years earlier.ConclusionsTHS responded well to steroid treatment, but if it was accompanied by optic neuritis as optic nerve involvement, we suggest follow-up with high dose steroid treatment especially was important.

Highlights

Tolosa-Hunt syndrome (THS) is an uncommon idiopathic granulomatous inflammatory disease of the cavernous sinus, superior orbital fissure, or orbit associated with ocular pain and ophthalmoplegia [1, 2]
All eleven patients had orbital or periorbital pain as the first symptom followed by diplopia or ptosis
Tolosa-Hunt syndrome is a diagnosis of exclusion requiring various examination and careful evaluation to rule out tumors, vascular causes, or other forms of inflammation in the lesion of the cavernous sinus and superior orbital fissure (Table 4)

Summary

Introduction

Tolosa-Hunt syndrome (THS) is an uncommon idiopathic granulomatous inflammatory disease of the cavernous sinus, superior orbital fissure, or orbit associated with ocular pain and ophthalmoplegia [1, 2]. The etiology of THS is still unknown. The diagnosis of THS should exclude other causes of painful ophthalmoplegia such as tumors, vasculitis, meningitis, sarcoidosis, diabetic ophthalmoplegia, and pseudotumor [3, 4]. THS is described in the International Classification of Headache Disorders (ICHD 3-beta) diagnostic criteria as. The objective of this study was to investigate the clinical features and outcomes of Tolosa-Hunt syndrome (THS)

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Conclusion