A 65-Year-Old Man With Diplopia and a Drooping Eyelid

Sumaira Nabi, MBBS; Sadaf Khattak, MBBS; Mazhar Badshah, MBBS, MD, FCPS

Disclosures

August 21, 2019

Discussion

The patient was given a trial of oral prednisolone and experienced significant pain relief within 48 hours. At discharge, after 10 days in the hospital, he had significant improvement in eye movement. He was discharged on a 6-week, tapering dose of oral steroids; the dosage was to be modified on follow-up visit based on his response.

Tolosa-Hunt syndrome (THS) is a rare disorder characterized by painful recurrent and relapsing ophthalmoplegia caused by nonspecific inflammation of the cavernous sinus and/or superior orbital fissure.[1] It manifests as recurrent episodes of retro-orbital, periorbital, or hemicranial pain, with ocular motor nerve palsies (ie, the third, fourth and sixth cranial nerves). Sensory loss in the distribution of the ophthalmic and occasionally the maxillary division of the trigeminal nerve may also be observed, occurring in various combinations and with oculosympathetic involvement.

In 1954, Tolosa reported THS for the first time, in a patient with painful, left-sided ophthalmoplegia, with involvement of left CN V1.[2] Cerebral angiography revealed segmental narrowing of the carotid siphon. Postmortem surgical exploration disclosed granulomatous inflammation of the left cavernous sinus and carotid artery. In 1961, Hunt et al described similar findings in 6 patients; they also reported the therapeutic efficacy of systemic corticosteroids, with prompt, dramatic improvement of signs and symptoms occurring in two of the patients.[3] In 1966, Smith and Taxdal termed this clinical condition Tolosa-Hunt syndrome.[4]

An uncommon disorder, THS affects males and females equally and can occur at virtually any age, although it is rarely seen during the first two decades of life.[1] Almost all patients report pain, which is a characteristic feature of the disease, although the nature of it is described differently by different patients. Usually, however, the pain is described as severe, sharp, stabbing, boring, lancinating, or intense. It may be periorbital in location, but some patients may report pain in different locations involving the retro-orbital, frontal, and temporal regions. In some cases, a severe hemicranial pain may be described. If left untreated, this pain persists, on average, for eight weeks. Either side may be affected; bilateral and/or simultaneous involvement may be present. The etiology of THS remains unknown.

Simultaneous onset of pain and ocular motor cranial nerve involvement may be noted, or a lag period as long as 2 weeks may occur between the two clinical features.[5] The oculomotor, trochlear, and abducens nerves may be involved alone or in various combinations. The pupil may have a normal size and reaction, as in our patient, or pupillary dilatation may occur secondary to parasympathetic third nerve involvement or constriction due to sympathetic involvement or Horner syndrome. Occasional optic nerve (CN II) involvement has been reported with THS if the pathologic process affects the orbital apex.[6,7,8] Variable degrees of visual loss may occur if optic nerve dysfunction is present. In rare cases, cranial nerves not located within the cavernous sinus/superior orbital fissure, including the mandibular division of the trigeminal nerve and the facial nerve, may be affected.[6,9,10] Other than nausea and vomiting, systemic or other neurologic involvement is not generally reported.

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