History
Patients present with usually severe retro-orbital or periorbital pain of acute onset. This pain may be described as constant and "boring" in nature.
Diplopia related to ophthalmoparesis follows the onset of pain (in rare cases, the ophthalmoparesis precedes the pain, sometimes by several days).
Patients may report visual loss. This is noted if the inflammation extends into the orbit to affect the optic nerve, and it is not a factor in disease limited to the cavernous sinus.
Paresthesias along the forehead may be described if the first division of the trigeminal nerve is involved.
Tolosa-Hunt syndrome is most often unilateral, although bilateral cases have been described.
Tolosa-Hunt syndrome frequently mimics other conditions; a single characteristic that is pathognomonic for this process does not exist. As such, realizing that this is a diagnosis of exclusion becomes even more important. Many of the processes that are found within the differential diagnosis of Tolosa-Hunt syndrome can have significant associated morbidity if not diagnosed and treated appropriately.
Physical
Painful ophthalmoparesis or ophthalmoplegia is the hallmark of this syndrome.
In addition to the optic and trigeminal nerves (V1, rarely V2 distribution), any of the ocular motor nerves may be involved. The oculomotor and abducens nerves are most commonly affected. Evidence of incomplete third nerve palsy with or without pupillary sparing may be present. Conversely, inflammatory involvement of the sympathetic nerves passing through the interior of the cavernous sinus may produce Horner syndrome with miosis. The combination of unilateral oculomotor palsy and Horner syndrome increases the localization specificity for the cavernous sinus.
Ptosis may be observed related to oculomotor palsy. Lid swelling is more likely to occur with orbital disease rather than inflammation limited to the cavernous sinus. These changes have been misdiagnosed as a complication of sinusitis, as reported by Lachanas et al. [4]
Mild proptosis and/or optic disc edema may be noted if the orbit is involved.
Evidence of trigeminal nerve involvement is suggested by loss of the ipsilateral corneal reflex.
The International Headache Society criteria for Tolosa-Hunt syndrome [5, 6] include the following:
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Episode(s) of unilateral orbital pain for an average of 8 weeks if left untreated
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Associated paresis of the third, forth, or sixth cranial nerves, which may coincide with onset of pain or follow it by a period of up to 2 weeks
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Pain that is relieved within 72 hours of steroid therapy initiation
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Exclusion of other conditions by neuroimaging and (not compulsory) angiography
Causes
The cause of Tolosa-Hunt syndrome is unknown (idiopathic).
The COVID-19 (SARS-CoV-2) pandemic has had a widespread impact across all specialties. At this time, there are no reported cases of Tolosa-Hunt syndrome associated with SARS-CoV-2. However, the possibility of thrombosis associated with SARS-CoV-2 is well described, and there has been a case report of superior ophthalmic vein thrombosis caused by COVID-19. As such, patients who present with painful ophthalmoplegia should be tested for SARS-CoV-2. [7]
Complications
Complications of high-dose and/or prolonged steroid use are common.
In patients with extracavernous sinus involvement affecting the optic nerve, loss of vision may occur.
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MRI of a 40-year-old man with severe periorbital pain ocular sinister (OS; ie, left eye), complete oculomotor nerve palsy OS, and partial abducens nerve palsy OS. Axial imaging without (left) and with (right) enhancement demonstrates nonspecific fullness involving the left cavernous sinus, consistent with Tolosa-Hunt syndrome within the context of the history. Treatment with steroids produced complete resolution of symptoms. Image courtesy of Eric Eggenberger, DO.
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Coronal T1-weighted MRI with (below) and without (above) enhancement demonstrates left cavernous sinus fullness consistent with Tolosa-Hunt syndrome (THS). The imaging features are nonspecific and must be placed into the context of the history, examination, and clinical course to avoid misdiagnosis of infiltrating, infectious, or neoplastic cavernous sinus processes. Image courtesy of Eric Eggenberger, DO.
