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Year : 2021  |  Volume : 1  |  Issue : 4  |  Page : 785-787

Recurrent alternating Tolosa-Hunt syndrome mimicking idiopathic hypertrophic pachymeningitis

Mahatma Gandhi Medical College and Research Institute, Pondicherry, India

Date of Submission21-Oct-2020
Date of Acceptance04-Mar-2021
Date of Web Publication09-Oct-2021

Correspondence Address:
Dr. Muthukrishnan Vallinayagam
Mahatma Gandhi Medical College and Research Institute, Pillayarkuppam, Pondicherry - 607 402
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_3309_20

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Tolosa–Hunt syndrome (THS) is a granulomatous inflammation in cavernous sinus or orbital apex. Recurrences are encountered ipsilaterally. Contralateral involvement is known as alternating THS. A middle-aged man presented with right sided headache and vomiting. Magnetic resonance imaging (MRI) revealed hyperintense lesion in right orbital apex with inflammatory changes in superior orbital fissure. A diagnosis of idiopathic hypertrophic pachymeningitis (IHP) was entertained. Within 48 h, there was complete ptosis and markedly restricted ocular movements. MRI done 3 months prior revealed similar features in left eye. A rare case of recurrent alternating THS with radiological evidence is portrayed here.

Keywords: Alternating THS, idiopathic hypertrophic pachymeningitis (IHP), recurrent THS, Tolosa–Hunt syndrome (THS)Tolosa–Hunt syndrome

How to cite this article:
Vallinayagam M, Deepikadevi S N, Pokal U, Balsamy M. Recurrent alternating Tolosa-Hunt syndrome mimicking idiopathic hypertrophic pachymeningitis. Indian J Ophthalmol Case Rep 2021;1:785-7

How to cite this URL:
Vallinayagam M, Deepikadevi S N, Pokal U, Balsamy M. Recurrent alternating Tolosa-Hunt syndrome mimicking idiopathic hypertrophic pachymeningitis. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2021 Oct 18];1:785-7. Available from: https://www.ijoreports.in/text.asp?2021/1/4/785/327669

Tolosa–Hunt syndrome (THS) is a nonspecific granulomatous inflammation in the cavernous sinus or superior orbital fissure typified by severe unilateral periorbital headache and ocular motor nerve paralysis. An overlapping prodrome of neurological features may mimic idiopathic hypertrophic pachymeningitis (IHP).[1],[2],[3] Synonymous with recurrent painful ophthalmoplegia, THS is known for recurrences which tend to be ipsilateral.[2] A young man with recurrent THS in the contralateral eye is discussed in this report.

  Case Report Top

A 35-year-old man presented with acute onset of severe right-sided headache and periorbital pain associated with vomiting for 1 week. There was no history of fever, drowsiness, or seizures. Visual acuity was unaffected. Corneal sensation, facial sensation, pupillary reactions, and fundus examination were normal. Systemic neurological examination was unremarkable. Examination of left eye revealed no abnormalities. There was past history of hospitalization 3 months back for a neurological illness.

MRI (T1-weighted fat saturated) revealed hyperintense lesion in the right orbital apex with inflammatory changes in superior orbital fissure and mild prominence of superior ophthalmic vein (T2 STIR) [Figure 1]. Magnetic resonance angiography (MRA) and magnetic resonance venography (MRV) failed to reveal any abnormality. A diagnosis of pachymeningitis was made and treatment was initiated with systemic antibiotics and low-dose corticosteroids in the medical ward.
Figure 1: MRI (T1 weighted fat saturated, axial section) revealing hyperintense lesion in the right orbital apex with inflammatory changes in superior orbital fissure (yellow arrow) and T2 STIR coronal section showing mild prominence of superior ophthalmic vein (red arrow)

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Within a span of 48 h, the patient presented with complete ptosis and restriction of ocular movements in all gazes in the right eye [Figure 2]. The restriction was less marked in down gaze than up gaze. The patient then related this to a similar episode in left eye 3 months back. There were no systemic neurological deficits. Prior medical records disclosed a diagnosis of orbital apex syndrome which resolved with systemic corticosteroids. The previous MRI done 3 months back (T2 FLAIR sequence) revealed an ill-defined hyperintense lesion at the left orbital apex and cavernous sinus, with thickening and enhancement of meninges in the left sylvian cistern overlying the fronto-temporal region [Figure 3]. Considering the classical full-blown clinical presentation in the current episode and MRI on two occasions, a presumptive diagnosis of THS was considered.
Figure 2: Restriction of ocular movements in the right eye (marked in up gaze)

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Figure 3: MRI (T2 FLAIR sequence) revealing an ill-defined hyperintense lesion at the left orbital apex and cavernous sinus (yellow arrow), with thickening and enhancement of meninges in the left sylvian cistern overlying the fronto-temporal region (green arrow) and superior ophthalmic vein enhancement (blue arrow)

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ESR was 52 mm and other routine hematological investigations were normal. CSF study was normal. Chest radiograph was normal. Systemic workup for immunological disorders and serological tests for HIV and VDRL were negative. A diagnosis of THS was made and was treated with IV methyl prednisolone followed by oral prednisolone, which was gradually tapered. The patient showed partial but dramatic resolution of ptosis and motility restriction within 48 h of initiating therapy [Figure 4]. On the basis of clinico-radiological features and an exquisite response to corticosteroids, recurrent alternating THS was confirmed.
Figure 4: Resolution of motility restriction in right eye following corticosteroid therapy

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  Discussion Top

The clinical diagnosis of THS includes unilateral orbital pain followed by ophthalmoplegia, secondary to granulomatous inflammation in the cavernous sinus, superior orbital fissure, or orbit. MRI or tissue biopsy is confirmatory.[4] This idiopathic entity virtually affects any age group with no gender predilection.[5] It is usually unilateral with no predominance on the basis of left and right eye involvement.[4],[5] A relapsing and remitting course is the norm, with attacks recurring every few months or years.[5] Recurrences are generally ipsilateral.[6]

The stabbing periorbital pain precedes ophthalmoplegia by a period of 2 weeks.[4],[5] Varying amalgamations of ocular motor nerve involvement have been reported, oculomotor nerve being the most common, followed by abducens, ophthalmic branch of trigeminal and trochlear nerve.[4] Trigeminal nerve is involved in 30% cases.[4],[5] Corneal sensation and facial sensation were normal, signifying sparing of trigeminal nerve in this patient.

Headache and ocular cranial nerve palsy may be the cardinal manifestations of IHP.[1] This overlapping clinical picture between IHP and THS may pose a diagnostic equivocacy. Whether the dual granulomatous entities exhibit clinically indistinguishable manifestations or whether they form different spectrum of a single entity is still ongoing research.[2]

Recurrent episodes of THS are common in younger patients and occur ipsilaterally. Contralateral ocular involvement in THS is rare and is known as alternating THS.[6] In a review of 233 cases, only two exceptional cases demonstrate alternating THS substantiated on imaging.[3] THS recurring in the fellow eye of a 24-year-old woman over a span of 4 months, with normal MRA and MRV picture and prompt resolution with corticosteroids is reported.[7] THS demonstrating a similar clinical behavior was observed in this patient.

An exhaustive array of differential diagnoses including vascular, infectious, immune and neoplastic etiology have to be excluded in order to clinch the diagnosis of THS. Microvascular infarcts secondary to diabetes, ophthalmoplegic migraine and non-specific orbital inflammation are other significant masquerades. A diagnosis of THS requires neuroimaging and thorough laboratory evaluation to exclude aforementioned entities.[8] Near-normal findings on imaging or mild enhancement in orbital apex coupled with enhancement of superior ophthalmic vein was suggestive of THS in this patient.

Dramatic resolution and pain relief is usually seen after 24–72 h of corticosteroid therapy.[8],[9] The presence of cranial nerve palsy portends good response to corticosteroid and complete resolution is anticipated in few weeks.[10] This patient exhibited a phenomenal response to corticosteroid therapy in 48 h.

  Conclusion Top

THS is a diagnosis of exclusion. A vigilant elimination of simulating entities by extensive imaging and serological evaluation is obligatory. THS is a nosological entity which can mimic or be one of the myriad manifestations of IHP. Contralateral eye involvement in a recurrent episode of THS is an extremely unusual clinical presentation.

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

Muthukumar N, Senthilbabu S, Usharani K. Idiopathic hypertrophic cranial pachymeningitis masquerading as Tolosa-Hunt syndrome. J Clin Neurosci 2005;12:589-92.  Back to cited text no. 1
Miwa H, Koshimura I, Mizuno Y. Recurrent cranial neuropathy as a clinical presentation of idiopathic inflammation of the dura mater: A possible relationship to Tolosa-Hunt syndrome and cranial pachymeningitis. J Neurol Sci 1998;154:101-5.  Back to cited text no. 2
Lekhra OP, Patel K, Amit M, Rathore Y. Alternating, recurrent Tolosa–Hunt syndrome –A rare case report. IOSR J Dent Med Sci 2013;4:1-4  Back to cited text no. 3
Headache Classification Committee of the International Headache Society (IHS). The international classification of headache disorders, 3rd edition (beta version). Cephalalgia 2013;33:629-808.  Back to cited text no. 4
Iaconetta G, Stella L, Esposito M, Cappabianca P. Tolosa-Hunt syndrome extending in the cerebello-pontine angle. Cephalalgia 2005;25:746-50.  Back to cited text no. 5
Kline LB, Hoyt WF. The Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiatry 2001;71:577-82.  Back to cited text no. 6
Hunt W, Meagher J, LeFever H, Zeman W. Painful ophthalmoplegia: Its relation to indolent inflammation of the cavernous sinus. Neurology 1961;11:56-62.  Back to cited text no. 7
Gladstone J, Dodick D. Painful ophthalmoplegia: Overview with a focus on tolosa-hunt syndrome. Curr Pain Headache Rep 2004;8:321-9.  Back to cited text no. 8
Navi BB, Safdieh JE. Recurrent, alternating Tolosa-Hunt syndrome. Neurologist 2010;16:54-5.  Back to cited text no. 9
Smith JL, Taxdal DS. Painful ophthalmoplegia. The Tolosa-Hunt syndrome. Am J Ophthalmol 1966;61:1466-72.  Back to cited text no. 10


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


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