Tolosa-Hunt syndrome: MR imaging features in 15 patients with 20 episodes of painful ophthalmoplegia

doi:10.1016/j.ejrad.2007.11.034

European Journal of Radiology

Volume 69, Issue 3, March 2009, Pages 445-453

https://doi.org/10.1016/j.ejrad.2007.11.034 Get rights and content

Abstract

Purpose

(a) To assess MR features in patients with Tolosa-Hunt syndrome (THS) and to (b) correlate MR findings with criteria derived from previously reported pathologic observations.

Methods

Fifteen patients with twenty episodes of painful ophthalmoplegia prospectively selected according to International Headache Society (IHS) standards underwent MR examinations focused on the cavernous sinus. Initial examinations in 20 and follow-up MR images in 17 episodes were retrospectively reviewed by 3 independent observers.

Results

The primary criteria: an enhancing soft tissue lesion within the cavernous sinus, increase in size and lateral bulging of the anterior cavernous sinus contour were consistently present in 15 initial episodes and in 5 recurrences (20/20). Agreement among observers was 100%. The secondary criteria: internal carotid artery narrowing in 7 patients, extension towards the superior orbital fissure in 13 and orbital apex involvement in 8 patients were unanimously agreed upon in 87.5%, 86.6% and 80%. Complete resolution of findings was observed on follow-up studies.

Conclusion

In patients with THS the MR features conform to previously reported pathologic findings. MR features are evocative of THS when an increase in size and bulging of the dural contour of the anterior CS supplemented by carotid artery involvement and extension towards the orbit are present. Resolution of findings within 6 months is required to support the diagnosis.

Introduction

Tolosa-Hunt syndrome (THS) is a painful ophthalmoplegia characterized by unilateral (peri-) orbital pain and oculomotor paresis with prompt relief of symptoms to steroid treatment. About 40% of patients experience ipsi- or contralateral relapse [1]. The clinical features and pathological findings were first described in a patient by Tolosa in 1954 [2] and substantiated with six additional cases by Hunt et al. in 1961 [3]. Histology in Tolosa's patient disclosed a non-specific periarteritic granuloma within the cavernous sinus, thickening of the perineurium and focal inflammation of the dural border of the cavernous sinus. In 1966 the eponym “Tolosa-Hunt syndrome” was coined by Smith and Taxdal [4] in a report of five new cases with emphasis on the use of steroids as a diagnostic test.

The 1988 classification of the International Headache Society (IHS) included THS among the cranial neuralgias [5]. Neuro-imaging was attributed the role of “exclusion of other causative lesions” like, e.g. neoplasm, metastasis, meningioma, lymphoma and infection or internal carotid artery aneurysm. Studying a series of 23 patients with painful ophthalmoplegia by MRI, Cakirer [6] confirmed THS in 5 five cases (21.7%), found alternative pathologies in 15 patients (65.2%) while in three cases (13%) no pathology was seen.

The ability to depict particular intracavernous findings by high resolution CT in two of five patients [7] and MR in nine out of eleven patients [8] implied that THS was “not necessarily a diagnosis of exclusion” any more [7]. Consequently the criteria of the 2004 IHS classification [9] were modified (Table 1) and “demonstration of a granuloma either by MR or biopsy” was added as diagnostic criterium. Based on the new classification, however, only 44 patients out of 124 patients (35%) compiled in a meta-analysis [10] fulfilled the revised IHS criteria by neuroimaging or biopsy confirmation.

The objective of this study is to analyze the MR findings in 15 patients with a total of 20 episodes of THS investigated by high resolution MR imaging specifically focused on the cavernous sinus and orbit. The MR features are studied in the light of previously reported pathologic findings and the revised 2004 IHS classification.

Section snippets

Patients and methods

Fifteen patients with the clinical diagnosis of THS were included into the study. Another four patients initially diagnosed as THS were excluded because of alternative imaging findings (meningioma, cavernous hemangioma, posterior communicating artery aneurysm and invasive macroadenoma). The 15 patients had been investigated between 1992 and 2006 and were prospectively followed by a neuro-ophthalmologist (K.L). Five patients presented with a recurrence within the study period. The clinical

Clinical findings

The clinical findings are summarized in Table 3. The mean age of patients (12 females, 3 males) at initial presentation was 57 years, the age range 23–75 years. Five patients developed a recurrence between 3 and 24 months, on average 14.2 months after the first episode. Painful ophthalmoplegia was the principal finding in every patient on initial presentation as well as on recurrence. Patients received oral treatment with 1–1.5 mg/kg of prednisone daily which was subsequently reduced based on

Discussion

As clinical features are suggestive but not specific for THS syndrome MR plays an essential role in establishing the diagnosis. The present study of 15 patients with 20 episodes of THS showed that MR imaging is capable of confirming the clinical diagnosis prospectively. In accordance with the location of symptoms and clinical findings an increase in size of the cavernous sinus was noted, which was reversible on follow-up examinations. Full agreement was between the three independent reviewers

Conclusion

High-resolution contrast-enhanced MR imaging directed to the cavernous sinus and orbit enables direct visualization and consequently recognition of the inflammatory tissue responsible for THS. In accordance with previous pathologic findings granulomatous tissue causes cavernous sinus enlargement, frequently involves the dural wall and tends to affect the internal carotid artery, superior orbital fissure and orbital apex. MR findings are evocative though not pathognomonic and should be

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Tolosa-Hunt syndrome: MR imaging features in 15 patients with 20 episodes of painful ophthalmoplegia

Abstract

Purpose

Methods

Results

Conclusion

Introduction

Section snippets

Patients and methods

Clinical findings

Discussion

Conclusion

Mayo Clin Proc

Surg Neurol

Radiol Clin N Am

The Tolosa-Hunt syndrome

J Neurol Neurosurg Psychiatry

Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoid aneurysm

J Neurol Neurosurg Psychiatry

Painful ophthalmoplegia: its relation to indolent inflammation of the cavernous sinus

Neurology

Painful ophthalmoplegia: the Tolosa-Hunt syndrome

Am J Ophthalmol

Classification and diagnostic criteria: classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain

Cephalalgia

MRI findings in the patients with the presumptive clinical diagnosis of Tolosa-Hunt syndrome

Eur Radiol

Tolosa-Hunt Syndrome revisited

AJF

MR imaging in Tolosa-Hunt syndrome

Am J Neuroradiol

The international classification of headache disorders ICHD-II

Cephalalgia

Tolosa-Hunt syndrome: critical literature review based on IHS 2004

Cephalalgia

Tolosa-Hunt syndrome: a case resort. Clinical and magnetic resonance imaging findings

J Neuroradiol

Idiopathic inflammatory pseudotumor of the orbit and Tolosa-Hunt syndrome—are they the same disease?

J Neurol

Painful ophthalmoplegia: overview with a focus on Tolosa-Hunt syndrome

Curr Pain Headache Rep

Gadopentetate dimeglumine-enhanced MR in the diagnosis of the Tolosa-Hunt syndrome

Am J Neuroradiol

Superior orbital fissure syndrome: report of a case caused by local pachymeningits

Arch Neurol

Orbital and retro-orbital pseudo-tumours

Mod Probl Ophthalmol