Tolosa-Hunt syndrome is a rare, idiopathic granulomatous inflammation of the cavernous sinus, superior orbital fissure, or orbit that leads to unilateral headache and orbital pain with diplopia and ipsilateral paresis of cranial nerves III, IV, VI, and/or the superior branches of V. Occasionally, vision can be affected. Patients may present at any age. This condition is typically unilateral but can be bilateral in 5% of cases.
Symptoms are very responsive to glucocorticoids but tend to relapse and remit if left untreated. The syndrome can resolve spontaneously or result in permanent neurologic damage.