Tolosa-Hunt Syndrome With Highly Elevated Interleukin-6 Levels

Tolosa-Hunt syndrome is a rare disorder of idiopathic granulomatous orbital inflammation associated with ipsilateral orbital pain and cranial nerve palsy.  The patient is a 42-year-old Hispanic female with Ecuadorian ancestry who presented in remission of episodes of horizontal diplopia, left eye proptosis, and 8/10 sharp left eye pain that occurred 10 years ago and four years ago. Both episodes resolved with about five weeks of oral methylprednisolone dosing prescribed at onset of symptoms. She is successfully maintained on oral methylprednisolone six-day dose pack about twice yearly for moderate orbital pain. At the time of the diplopia four years ago, Magnetic Resonance Imaging showed a left cavernous sinus mass, which resolved on subsequent imaging. Her interleukin 6 (IL-6) serum level done during remission and during clinical episodes was highly elevated at 90.5 pg/ml (normal <5 pg/ml) and 68.7 pg/mL, respectively. Lower level during clinical episode may be due to use of oral steroid medication. The cytokine elevation may provide clues regarding the pathophysiology of this unusual neurological disorder.