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 Table of Contents  
Year : 2023  |  Volume : 3  |  Issue : 1  |  Page : 139-141

Unilateral Tolosa–Hunt syndrome with bilateral cavernous sinus involvement – A very rare presentation

1 Department of Ophthalmology, Adesh Medical College and Hospital, Haryana, India
2 Department of Ophthalmology, SRMS Medical College and Hospital, Bareilly, Uttar Pradesh, India
3 Department of Radiology, Adesh Medical College and Hospital, Mohri, Kurukshetra, Haryana, India

Date of Submission29-Jul-2022
Date of Acceptance14-Oct-2022
Date of Web Publication20-Jan-2023

Correspondence Address:
Sujit Das
Department of Ophthalmology, SRMS Medical College and Hospital, Bareilly - 243202, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_1862_22

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Tolosa–Hunt syndrome (THS) is a rare, idiopathic, inflammatory process of the cavernous sinus and remains a diagnosis of exclusion. It is characterized by unilateral orbital pain and is often associated with diplopia due to the involvement of one or more oculomotor nerves. Involvement of the cranial nerve V, cranial nerve VII, or the ipsilateral sympathetic pupillary pathway, has also been described. Bilateral cranial nerve involvement is very infrequent. Herein, we are presenting a case of unilateral THS with bilateral cavernous sinus involvement.

Keywords: Orbital apex syndromes, painful ophthalmoplegia, ptosis, third nerve palsy, Tolosa–Hunt syndrome

How to cite this article:
Das S, Mehrotra N, Aggarwal A. Unilateral Tolosa–Hunt syndrome with bilateral cavernous sinus involvement – A very rare presentation. Indian J Ophthalmol Case Rep 2023;3:139-41

How to cite this URL:
Das S, Mehrotra N, Aggarwal A. Unilateral Tolosa–Hunt syndrome with bilateral cavernous sinus involvement – A very rare presentation. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Feb 1];3:139-41. Available from: https://www.ijoreports.in/text.asp?2023/3/1/139/368183

Tolosa–Hunt syndrome (THS) is a rare neurological condition of unknown etiology and characterized by unilateral orbital pain and ipsilateral oculomotor nerve paralysis. It is caused by a non-specific inflammation in the region of the cavernous sinus and the sphenoid cleft.[1] It is a very rare syndrome with an estimated annual incidence of one case per million per year.[2] In most cases, the individual experiences intense pain and paralysis of the extraocular muscles.[3] In addition, affected individuals may experience paralysis of the facial nerve also.[4] Although considered a benign condition, permanent neurologic deficits can occur, and relapses are common. It often requires prolonged immunosuppressive therapy.[5],[6]

  Case Report Top

A 52-year-old female patient presented with complaints of drooping of her right upper lid with pain and diplopia of 7 days onset [Figure 1]a. She was suffering from on-and-off right-sided throbbing headache and orbital pain for the last few days followed by a sudden onset of moderate ptosis. There was no preceding history of any fever or systemic illness. There was no family history of migraine and hypertension. Her blood pressure was 128/77 mmHg. In both eyes, the vision was 6/9 and intraocular pressure was 14 mmHg. She had 15-degree exotropia in the right eye [Figure 1]b. The pupillary size and reaction were normal. In the right eye, there were limitations of adduction [Figure 2]a, elevation [Figure 2]b, abduction [Figure 2]c, and depression [Figure 2]d, suggesting third and sixth nerve paresis. Corneal sensation was reduced in the right along with moderate ocular tenderness. The rest anterior and posterior segment examination was normal. There was no sign of facial nerve palsy. The left eye examination was absolutely normal. Considering an inflammatory etiology involving the cavernous sinus, magnetic resonance imaging (MRI) was done along with a complete blood count, ESR [erythrocyte sedimentation rate], C-reactive protein, and chest X-ray. Ear Nose Throat [Otolaryngology] (ENT) opinion was taken and no abnormality was detected. A neurological opinion was also taken and MRI angiography of the brain was advised. Due to a lack of financial issues, she could not do an MRI angiography. Based on clinical findings, a provisional diagnosis of THS was made and she was started on oral steroid 60 mg/day [1 mg/k/bwt]. She responded well within 24 h with no pain. On an MRI scan, an axial T2-weighted image showed heterogeneity with soft tissue density in the cavernous sinus bilaterally [Figure 3]a. Axial and coronal fluid attenuated inversion recovery (FLAIR) images showed hyperintensity in the cavernous sinus bilaterally [Figure 3]b. Coronal T1 FS post-contrast image showed accentuated enhancement and no filling defect in the cavernous sinus bilaterally [Figure 3]c. The optic nerve [Figure 4]a, para nasal sinuses [Figure 4]b, orbital apex [Figure 4]c and the rest of the brain parenchyma [Figure 4]d appeared normal on the MRI scan. Her ESR report was slightly high [31 mm/h] and her C-reactive protein level was 117.
Figure 1: (a) Right eye ptosis. (b) Right eye exotropia on Hirschberg's test. (c) Ptosis improvement with a high dose of steroids

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Figure 2: (a) Limitation of right eye medial rectus movement. (b) Limitation of right eye superior rectus movement. (c) Limitation of right eye lateral rectus movement. (d) Limitation of right eye inferior rectus movement

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Figure 3: (a) Axial T2-weighted image showing heterogeneity with soft tissue density in cavernous sinus bilaterally. (b) Axial and coronal FLAIR images showing hyperintensity in cavernous sinus bilaterally. (c) Coronal T1 FS post-contrast image showing accentuated enhancement and no filling defect in the cavernous sinus bilaterally

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Figure 4: (a) Optic nerve appears normal on MRI. (b) Paranasal sinus findings are normal on MRI. (c) The orbital apex appears normal on MRI. (d) Brain parenchyma appears normal on MRI

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Finally, a diagnosis of THS was documented in our case, and she was continued on oral steroids in tapering doses over a period of 6 weeks. After 7 days, there was an improvement in ptosis and the patient was symptomatically better on follow-up [Figure 1]c.

  Discussion Top

THS is considered a diagnosis of exclusion of painful ophthalmoplegia.[7] Bilateral cranial nerve involvement is very infrequent.[8] Patients should undergo thorough investigations to rule out other causes of symptoms. Routine complete blood count (CBC), ESR, C-reactive protein, chest radiography, serology, immunological tests, a lumbar puncture, and a neuroimaging study are essential primary investigations.[7],[8] Our patient presented with unilateral painful ophthalmoplegia with ptosis. Her ESR was slightly high [31 mm/h] and her C-reactive protein level was 117. MRI is useful in detecting inflammatory changes in the cavernous sinus, superior orbital fissure, and/or orbital apex. Inflammatory change in the orbit in the absence of cranial nerve palsy is described as an orbital pseudotumor.[9] The contribution of MRI, as an examination, is essential to eliminating other specific inflammatory processes such as lymphoma, meningioma, and sarcoidosis.[9] In our case, there was radiological evidence of non-specific inflammatory etiology in both the cavernous sinuses.

Sometimes, a biopsy may need to confirm the diagnosis, as it is useful in ruling out a neoplasm.[6],[8],[9] The following essentially clinical criteria have been established by Hunt.[5],[6] One or more episodes of unilateral orbital pain persisting for weeks without treatment or paresis of the third, fourth, and/or sixth cranial nerves and/or detection of granulomas by MRI or by biopsy or pain and paresis disappear within 72 h if treatment with corticosteroids is adequate, and finally other etiologies have been excluded by appropriate investigations.[5],[6]

In our context, there was an excellent response to corticosteroid therapy within 24 h, and to confirm the diagnosis of THS, a biopsy was not carried out considering the risk of complications. Kóbor et al.[9] recommend a repeat of the MRI in a patient with an initially normal MRI to reveal new inflammatory signs, which could appear months after the initial presentation. In addition, in agreement with Zhang et al.,[2] we also believe that the response to steroids is probably still important to support the diagnosis of THS in clinical practice, in the absence of other specific and reliable criteria. Treatment consists of a high dose of oral prednisone for 4 weeks. Significant improvement is often evident from the first 24h of treatment.[10] Our patient received oral prednisone 60mg per day and had a complete regression of the symptoms after a week. After more than 6 months of monitoring, no clinical abnormality was observed in our patient.

  Conclusion Top

Bilateral cranial nerve involvement is very infrequent in THS. We have presented a case of unilateral THS with bilateral cavernous sinus involvement, which responded well to systemic steroids.

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.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Mantia LL, Curone M, Rapoport A, Bussone G, Tolosa-hunt syndrome: Critical literature review based on IHS 2004 criteria. Cephalalgia 2006;26:772-81.  Back to cited text no. 1
Zhang X, Zhou Z, Steiner TJ, Zhang W, Liu R, Dong Z, et al. Validation of ICHD-3 beta diagnostic criteria for 13.7 Tolosa-Hunt syndrome: Analysis of 77 cases of painful ophthalmoplegia. Cephalalgia 2014;4:624-32.  Back to cited text no. 2
Chaudhary V, Venu S, Deswal J. Painful ophthalmoplegia due to Tolosa-Hunt syndrome: A case report. Int J Med Sci Public Health 2016;5:1045-9.  Back to cited text no. 3
Kontzialis M, Choudhri AF, Patel VR, Subramanian PS, Ishii M, galia GL, et al. High resolution 3D Magnetic rsonance maining of the sixth Cranial Nerve. Jounal of Neuro-Opthalmol. Dec 2015;35:412-25.  Back to cited text no. 4
Singh MK, Marshall B, Hawley J. Painful ophthalmoplegia: A case of tolosa-hunt syndrome. Mi Medi 2014;179:1409-10.  Back to cited text no. 5
Rousseaux P, Perruzi P, Schaiso-cusin M. Should Tolosa-Hunt syndrome still be an exclusion diagnosis? Rev Neurol 1993;149:315-9.  Back to cited text no. 6
Mandrioli J, Frank G, Sola P, Leone ME, Guaraldi G, Guaraldi P, et al. Tolosa-hunt syndrome due to actinomycosis of the cavernous sinus: The infectious hypothesis revisited. Headache 2004;44:806-11.  Back to cited text no. 7
Kline LB, Hoyt WF. Nosological entities?: The Tolosa-Hunt syndrome. J Neurol, Neurosurg Psychiatry 2001;71:577-82.  Back to cited text no. 8
Kóbor J, Vörös E, Deák A. Magnetic resonance imaging in Tolosa-Hunt syndrome. Eur J Pediatr 2004;163:753.  Back to cited text no. 9
Drouot X, Brosset C, Sagui E, Bregigeon M. Le syndrome de Tolosa-Hunt: Apport de l'imagerie actuelle. Rev Méd Intern 2002;23:479-81.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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