Clinical Observations on Tolosa-Hunt Syndrome

Han Kee Lee; Sul Gee Lee

doi:10.3341/jkos.2009.50.11.1717

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Lee and Lee: Clinical Observations on Tolosa-Hunt Syndrome

Original Article

J Korean Ophthalmol Soc 2009;50(11):1717-1723.

Published online: 31 August 2009

DOI: https://doi.org/10.3341/jkos.2009.50.11.1717

Clinical Observations on Tolosa-Hunt Syndrome

Han Kee Lee, MD, Sul Gee Lee, MD, PhD

Department of Ophthalmology, College of Medicine, Inje University, Busan, Korea, Department of Ophthalmology, Research Foundation, Inje University, Busan, Korea

Address reprint requests to Sul Gee Lee, MD, PhD, Department of Ophthalmology, College of Medicine, Inje University #633-165 Gaegeum-dong, Busanjin-gu, Busan 614-735, Korea Tel: 82-51-890-6016, Fax: 82-51-890-6329, E-mail: judysg@hanmail.net

Received 27 April 2009 Accepted 28 July 2009

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

The authors reviewed clinical features, response to treatment and recurrence rate of Tolosa-Hunt syndrome.

Methods

A retrospective chart review was performed on 6 patients, who fulfilled the diagnosis for Tolosa-Hunt syndrome according to the International Headache Society (IHS) classification of 2004.

Results

Every patient had orbital pain as a first symptom, followed by cranial nerve paresis. The third cranial nerve was most commonly involved (83.3%), followed by the sixth nerve (50%), the forth nerve (16.7%), and the first branch of the fifth cranial nerve (16.7%). Two of the patients showed multiple cranial nerve paresis (33.3%, 2 out of 6). All patients received high-dose steroid therapy for more than 5 days, and all patients had resolution of orbital pain within 72 hours of treatment. Full recovery of cranial nerve paresis occurred on average in 2.3 months (3 days to 12 months). During the 29 months of follow-up, 2 patients (33.3%) had a recurrence episode.

Conclusions

Tolosa-Hunt syndrome responds well to steroid therapy, and full recovery is possible with proper treatment. The exact diagnosis and treatment of Tolosa-Hunt syndrome is important. Because Tolosa-Hunt syndrome often recurs after full recovery, the authors suggest a minimum follow-up period of 2 years.

Keywords: Painful ophthalmoplegia, Tolosa-Hunt syndrome

References

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Figure 1.

Photograph of case 2 showing ophthalmoplegia and mild ptosis in the left eye.

Figure 2.

T1-weighted MRI shows a contrast-enhancing lesion with undulated margin and thickening in the left anterior cavernous sinus and superior orbital fissure area (red arrow).

Figure 3.

Two weeks after steroid pulse therapy of case 2. Periocular pain and ptosis in the left eye disappeared, and left ophthalmoplegia showed much improvement.

Table 1.

Diagnostic criteria for Tolosa-Hunt syndrome (IHS classification of 2004)

A. One or more episodes of unilateral orbital pain persisting for weeks if untreated

B. Paresis of one or more of the third, fourth and/or sixth cranial nerves and/or demonstration of granuloma by MRI or biopsy

C. Paresis coincides with the onset of pain or follows it within 2 weeks

D. Pain and paresis resolve within 72 h when treated adequately with corticosteroids

E. Other causes have been excluded by appropriate investigations^*

^*Other causes of painful ophthalmoplegia include tumors, vasculitis, basal meningitis, sarcoid, diabetes mellitus and ophthalmoplegic ‘migraine’.

Table 2.

Clinical characteristics

Table 3.

Treatment response

Case	Pain resolution (hours)	Paralysis resolution (days)	Recurrence (month)
1	48	14	−
2	70	21	−
3	48	14	−
4	72	10	−
5	50	360	6
6	72	3	12

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