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 Table of Contents  
Year : 2018  |  Volume : 13  |  Issue : 2  |  Page : 141-142

Tolosa–Hunt syndrome in a patient of lupus

Department of Immunology and Rheumatology, Sir Ganga Ram Hospital, New Delhi, India

Date of Web Publication24-May-2018

Correspondence Address:
Dr. Jeet Patel
2208 A, SSRB, Sir Ganga Ram Hospital, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/injr.injr_2_18

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How to cite this article:
Patel J, Duggal L, Jain N, Singh BG, Basnet A, Bisht A. Tolosa–Hunt syndrome in a patient of lupus. Indian J Rheumatol 2018;13:141-2

How to cite this URL:
Patel J, Duggal L, Jain N, Singh BG, Basnet A, Bisht A. Tolosa–Hunt syndrome in a patient of lupus. Indian J Rheumatol [serial online] 2018 [cited 2022 Jun 28];13:141-2. Available from:

Dear Editor,

Tolosa–Hunt syndrome (THS) is a rare cause of painful ophthalmoplegia with idiopathic inflammatory lesions of retro-orbital structures, for example, cavernous sinus, orbital apex, or superior orbital fissure.[1] THS is one of the rare ophthalmic complications of systemic lupus erythematosus (SLE). We describe here a case of SLE complicated by glucocorticoids resistant THS which was then treated with injectable cyclophosphamide.

A 55-year-old female of SLE (SLE-SLICC classification criteria: mouth ulcer, synovitis, thrombocytopenia, leukopenia, antinuclear antibodies positive, and low complement) was adequately controlled with corticosteroids and disease modifying anti-rheumatic drugs. She presented with right-sided headache, diplopia, decreased vision in both eyes, and fever for 14 days. Ophthalmologic examinations revealed no pathologic findings. Magnetic resonance imaging (MRI) orbit showed mild thickening of right optic nerve with altered signal intensity and was suggestive of ischemic optic neuropathy/optic neuritis. She was given 1g methylprednisolone pulse therapy for 3 days, followed by 1 mg/kg/day of prednisolone for the next 12 days. She did not improve and in addition, developed right 3rd, 4th, and 6th cranial nerve palsies at 29th day of the initial symptom. Repeat MRI orbit showed the presence of small ill-defined soft tissue in the region of orbital apex suggestive of possibility of THS [Figure 1]. In view of fever, computed tomography-guided biopsy of lesion was done to rule out infection/malignancy, and it revealed nonspecific inflammation and no evidence of fungus/mycobacterium tuberculae/mitosis. After ruling out infections, injection cyclophosphamide 500 mg was given. Vision improved with subsidence of fever and ophthalmoplegia totally recovered. Second dose of 500 mg injection cyclophosphamide was given after 15 days. Her vision continued to improve over the next 1 month. Azathioprine was started after 1 month of the last dose of cyclophosphamide. The patient gained full vision over the next 2 months after which she lost to follow-up.
Figure 1: Second magnetic resonance imaging orbit: Small ill-defined soft tissue in the region of orbital apex, suggesting possibility of Tolosa–Hunt syndrome

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THS is a rare idiopathic disorder occurring after 20 years of age without any sex predilection. Treatment still lacks standard guidelines and there is scarce data on the use of immunosuppressive agents. There are two reviews of THS based on two diagnostic criteria given by the International Headache Society and International Classification of Headache Disorders-II criteria.[2],[3] According to these criteria, clinical scenario of unilateral headache for >8 weeks duration, good response to glucocorticoids, MRI findings of inflammation in retro-orbital structures and/or granuloma on biopsy are required to classify THS. Few known associations and/or close differential diagnosis of autoimmune disorders are SLE, sarcoidosis, granulomatosis, polyangiitis etc., Varicella zoster, Lyme disease, central nervous system Whipple disease, and cytomegalovirus are among few infectious etiologies.[4] Usual clinical picture involves deep boring eye pain, unilateral continuous headache, double vision, vertigo, and visual disturbances. Spontaneous remissions have been noted, but relapses are quite common (40%–50%). Glucocorticoids are the drug of choice with response within 48–72 h albeit not all patients respond.[5] Although there are reports of azathioprine and methotrexate use in few corticosteroid-resistant patients in literature, none has used cyclophosphamide. This is the first case from India where injectable cyclophosphamide was used successfully in SLE-THS patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Hao R, He Y, Zhang H, Zhang W, Li X, Ke Y, et al. The evaluation of ICHD-3 beta diagnostic criteria for Tolosa-Hunt syndrome: A study of 22 cases of Tolosa-Hunt syndrome. Neurol Sci 2015;36:899-905.  Back to cited text no. 1
La Mantia L, Curone M, Rapoport AM, Bussone G; International Headache Society. Tolosa-Hunt syndrome: Critical literature review based on IHS 2004 criteria. Cephalalgia 2006;26:772-81.  Back to cited text no. 2
Colnaghi S, Versino M, Marchioni E, Pichiecchio A, Bastianello S, Cosi V, et al. ICHD-II diagnostic criteria for Tolosa-Hunt syndrome in idiopathic inflammatory syndromes of the orbit and/or the cavernous sinus. Cephalalgia 2008;28:577-84.  Back to cited text no. 3
Goadsby PJ, Lance JW. Clinicopathological correlation in a case of painful ophthalmoplegia: Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiatry 1989;52:1290-3.  Back to cited text no. 4
Zhang X, Zhang W, Liu R, Dong Z, Yu S. Factors that influence Tolosa-Hunt syndrome and the short-term response to steroid pulse treatment. J Neurol Sci 2014;341:13-6.  Back to cited text no. 5


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