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CASE REPORT
Year : 2020  |  Volume : 9  |  Issue : 4  |  Page : 259-263

The Tolosa-Hunt syndrome-Role of MR imaging


Departments of Radiology and Neurology, ESIC Superspeciality Hospital, Sanathnagar, Hyderabad, Telangana, India

Date of Submission04-Jun-2020
Date of Acceptance28-Jun-2020
Date of Web Publication6-Jan-2021

Correspondence Address:
Dr. Anurudh Kishore Vatti
ESIC Superspeciality Hospital, Sanathnagar, Hyderabad, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JDRNTRUHS.JDRNTRUHS_85_20

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  Abstract 


Tolosa–Hunt syndrome (THS) is a painful ophthalmoplegia due to nonspecific inflammation in the cavernous sinus (CS) and superior orbital fissure/orbital? apex. Diagnosis is based on clinical manifestations, magnetic resonance imaging (MRI) findings, rapid response to steroids, and exclusion of other causes of CS involvement. Our case report is regarding a 48-year-old female presenting with unilateral orbital pain, restricted ocular movements, and ptosis. The clinical presentation can be a pointer to several conditions of the CS and a correct diagnosis is a must to institute appropriate early management. This case is to emphasize the role of contrast-enhanced magnetic resonance imaging in the diagnosis of this condition and prompt clinical response to steroid therapy. This condition may be sight-threatening if untreated inflammation extends beyond CS to affect the optic nerve.

Keywords: Cavernous sinus, contrast enhanced-magnetic resonance imaging, ophthalmoplegia, orbital apex, Tolosa–Hunt syndrome


How to cite this article:
Vatti AK, Seelam S, Basireddy N, Vidavaluru VR. The Tolosa-Hunt syndrome-Role of MR imaging. J NTR Univ Health Sci 2020;9:259-63

How to cite this URL:
Vatti AK, Seelam S, Basireddy N, Vidavaluru VR. The Tolosa-Hunt syndrome-Role of MR imaging. J NTR Univ Health Sci [serial online] 2020 [cited 2021 Jan 23];9:259-63. Available from: https://www.jdrntruhs.org/text.asp?2020/9/4/259/306129




  Introduction Top


First described by Tolosa in 1954 in a patient with unilateral recurrent painful ophthalmoplegia with the involvement of cranial nerves III, IV, and VI, his findings of carotid angiogram showed narrowing of carotid siphon.[1],[2],[3] Hunt described similar features in six patients in 1961.[1] The entity was termed Tolosa–Hunt syndrome (THS) by Smith and Taxdol (1966).[4] Initially evaluated by cerebral angiography and orbital venography, the results yielded nonspecific findings. With the advent of CT and magnetic resonance imaging (MRI) direct visualization of the cavernous sinus (CS) is possible. Using these neuroimaging modalities, CS and orbital apex abnormalities have now been described in THS.


  Case History Top


A 48-year-old female presented with complaints of headache for 21 days and blurring of vision for 14 days. Headache was limited to the right frontotemporal region.

Heart rate (HR) and blood pressure (BP) were within normal limits. She had incomplete ptosis of the right eye. Ocular movements on the left side were normal. On the right side, all the ocular movements, abduction, adduction, elevation, and depression were restricted. No sensory loss over the face was detected. The motor system was normal. The patient underwent extensive laboratory workup, which was nonspecific.

The patient was referred for an MRI examination. Contrast-enhanced MRI was performed in a SIEMENS 3 T (Magnetom verio machine). Gadolinium was administered (0.1 mmol/kg). Pre- and post contrast coronal and axial contrast-enhanced T1-weighted fat saturated (FS CE-T1W) sequences were acquired.

MRI demonstrated abnormal enhancing soft tissue in the right CS, extending anteriorly through the superior orbital fissure into the orbital apex. All the extraocular muscles of the right eye (superior, inferior, medial, lateral rectus, and superior and inferior oblique muscles) and their tendinous insertion showed significant enlargement with abnormal enhancement [Figure 1],[Figure 2],[Figure 3],[Figure 4],[Figure 5],[Figure 6],[Figure 7],[Figure 8],[Figure 9]. No luminal narrowing of the internal carotid artery was demonstrated [Figure 5]. There was thickening and enhancement of the dura overlying right basifrontal lobe, anterior temporal lobe, and right petroclinoid fold [Figure 1], [Figure 2], [Figure 8] and [Figure 9]. Optic nerve sheath surrounding the intraorbital part of the right optic nerve showed thickening and enhancement.
Figure 1: Axial contrast-enhanced T1-weighted fat saturated (CE T1W FS) image at the level of orbit shows abnormal enlargement and enhancement of right lateral rectus muscle (blue arrow), enhancement of right temporal dura (pink arrow) and right petroclinoid fold (purple arrow)

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Figure 2: Axial CE T1W FS image at the level of orbit, shows abnormal enlargement and enhancement of right lateral rectus muscle (blue arrow) and enhancement of right temporal dura (pink arrow)

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Figure 3: Axial CE T1W FS image at the level of cavernous sinus shows abnormal soft tissue enhancement in the region of right cavernous sinus (purple arrow)

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Figure 4: Axial T2W FS image at the level of orbit, shows abnormal enlargement and altered signal intensity of right lateral rectus (blue arrow) and medial rectus muscle (green)

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Figure 5: Coronal CE T1W FS image at the level of cavernous sinus shows abnormal soft tissue enhancement in the region of right cavernous sinus (blue arrow). Right cavernous carotid artery shows normal caliber (green arrow)

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Figure 6: Coronal CE T1W FS image at the level of cavernous sinus shows abnormal soft tissue enhancement in the region of right cavernous sinus (arrow)

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Figure 7: Coronal CE T1W FS image shows abnormal soft tissue enhancement in the region of right orbital apex (arrow)

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Figure 8: Coronal CE T1W FS image at the level of orbit shows thickening and enhancement of tendinous insertion site of right extraocular muscles (black arrow) and right basifrontal dura (blue arrow)

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Figure 9: Coronal CE T1W FS image at the level of orbit shows thickening and enhancement of all the extraocular muscles of right eye

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The above imaging findings were consistent with THS within the context of clinical history.

The patient was treated with steroids (pulse methylprednisolone 1 g/day IV for 5 days). There was progressive relief of symptoms within the next 48 h. Clinical follow-up at 15 Days showed resolution of ptosis and restoration of ocular movements. Follow-up MRI done after 28 days showed complete resolution of the inflammation and significant improvement in the symptoms [Figure 10].
Figure 10: Axial CE T1W FS image (4 weeks posttreatment) shows complete resolution of the inflammation

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  Discussion Top


Defination of the syndrome: The syndrome of painful ophthalmoplegia consists of periorbital or hemicranial pain, combined with ipsilateral ocular motor nerve palsies, oculosympathetic paralysis, and sensory loss in the distribution of the ophthalmic, and occasionally, maxillary division of the trigeminal nerve. Various combinations of these cranial nerve palsies may occur, localizing the pathological process to the region of the CS/superior orbital fissure.

The constellation of findings described may be due to four major causes: trauma; neoplasm; aneurysm; and inflammation. Comprehensive patient evaluation is essential in establishing the correct diagnosis. Within the last diagnostic category, there is a specific subset of patients who develop painful ophthalmoplegia due to a nonspecific inflammatory process in the region of the CS/superior orbital fissure. Infrequently, they experience involvement of additional cranial nerves ipsilateral to the ophthalmoplegia, including the optic nerve, mandibular branch of the trigeminal nerve, and facial nerve. Having a relapsing and remitting course, they respond promptly to systemic corticosteroid therapy. The diagnostic eponym THS has been applied to these patients, and it is this entity, which forms the basis of our case report.

This syndrome is caused by a nonspecific inflammatory process in the CS or superior orbital fissure.[1],[2],[3] The inflammation causes extrinsic compression of the neurovascular structures that cross the CS including cranial nerves III, IV, V, and VI. Orbital venography is helpful to prove occlusion of the superior ophthalmic vein, but it is difficult to visualize the lesion in the CS.[5] CT scans are normal in the majority of patients with THS, few of them showed abnormal soft-tissue area in both the CS and orbital apex.[6],[7],[8] Nevertheless, the limitation of contrast resolution due to a lack of sensitivity to soft-tissue change with superimposed beam hardening and bony streak artifacts makes it difficult to accurately visualize the lesions in the CS. MRI can reveal localized minute lesions in the brain and is suited for assessing lesions surrounded by bone such as the CS and orbit.[9]

Gadolinium-enhanced MRI is the imaging modality of choice to evaluate THS. Diagnosis is based on clinical manifestations, MRI findings, rapid response to steroids, and exclusion of other causes of CS involvement.

MRI findings include abnormal enlargement and enhancement of the CS extending through the superior orbital fissure into the orbital apex. Enhancement may extend to involve the extraocular muscles. Reported MRI findings on T1-weighted and T2-weighted images are extremely variable and nonspecific, including iso- to hypointense signal in the region of the CS and orbital apex. Narrowing of the intracavernous portion of the internal carotid artery is seen in few cases. Involvement of optic nerve is seen in complicated cases.

MRI is important to rule out other pathological processes presenting with similar clinical features like meningioma, sarcoidosis, pituitary tumors, tuberculous meningitis, lymphoma, and aneurysms.

THS is essentially a clinical diagnosis of exclusion. Exclusion of other conditions by neuroimaging is important. Pain that is relieved within 48 hours of steroid therapy is characteristic.

MRI gives useful information for the diagnosis of THS and should facilitate monitoring the response to treatment.


  Conclusion Top


THS diagnosis is based on clinical manifestations, MRI findings, rapid response to steroids, and exclusion of other causes of CS involvement. The role of radiologists is to exclude other conditions causing similar clinical features.

CE-MRI plays a vital role in the early diagnosis of this sight-threatening disorder.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hunt WE, Meagher JN, LeFever HE, Zeman W. Painful ophthalmoplegia. Its relation to indolent inflammation of the cavernous sinus. Neurology 1961;11:56-62.  Back to cited text no. 1
    
2.
Hunt WE. Tolosa-Hunt syndrome: One cause of painful ophthalmoplegia. J Neurosurg 1976;44:544-9.  Back to cited text no. 2
    
3.
Tolosa E. Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoid aneurysm. J Neurol Neurosurg Psychiatry 1954;17:300-2.  Back to cited text no. 3
    
4.
Smith JL, Taxdal DSR. Painful ophthalmoplegia. The Tolosa-Hunt syndrome. Am J Ophthalmol 1966;61:1466-72.  Back to cited text no. 4
    
5.
Muhletaler CA, Gerlock AJ Jr. Orbital venography in painful ophthalmoplegia (Tolosa-Hunt syndrome). Am J Radiol 1979;133:31-4.  Back to cited text no. 5
    
6.
Aron-Rosa D, Doyon D, Salamon G, Michotey P. Tolosa- Hunt syndrome. Ann Ophthalmol 1978;10:1161-8.  Back to cited text no. 6
    
7.
Van Dalen JTW, Bleeker GM. The Tolosa-Hunt syndrome. Doc Ophthalmol 1977;44:167-72.  Back to cited text no. 7
    
8.
Kwan ESK, Wolpert SM, Hedges TR III, Laucella ML. Tolosa-Hunt syndrome revisited: Not necessarily a diagnosis of exclusion. Am J Neuroradiol 1987;8:1067-72.  Back to cited text no. 8
    
9.
Smith FW, Cherryman GR, Singh AK, Forrester JV. Nuclear magnetic resonance tomography ofthe orbit at 3.4 MHz. Br J Radiol 1985;58:947-57.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]



 

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