Tolosa-Hunt Syndrome with Highly Elevated Interleukin 6 Levels: Case Report

Background Ophthalmoplegic migraine was first defined by Charcot as "a condition associated with migraine in which there is a palsy of an ocular nerve, usually the third, temporary at first and later becoming permanent." Under current International Classification of Headache Disorders (ICHD) third edition criteria, ophthalmoplegic migraine requires absence of a mass lesions, whereas a granulomatous periorbital lesion is required for the diagnosis of Tolosa-Hunt syndrome which is considered to be idiopathic orbital inflammation and causes orbital pain with ipsilateral cranial nerve palsy. Most cases improve with steroids. Case report The patient is a 42-year-old Hispanic female with Peruvian ancestry who presented with episodes of diplopia, proptosis, and eye pain. She had a severe episode 10 years ago which led to horizontal diplopia as well as another episode 4 years ago. Both episodes resolved with about five weeks of oral methylprednisolone dosing. There is complete relief of diplopia between these episodes. She is maintained on oral methylprednisolone 6-day dose pack about twice yearly for orbital pain. At the time of the diplopia 4 years ago, Magnetic Resonance Imaging showed a left cavernous sinus mass, which resolved on subsequent imaging. Her Interleukin 6 (IL-6) serum level done outside of the episodes was highly elevated at 90.5 pg/ml (normal <5 pg/ml). Discussion The pathology of the original case of Dr. Eduardo Tolosa was reviewed by Hunt et al. in 1961. While the pathology of Tolosa-Hunt syndrome is generally described as granulomatous, the slides showed a lymphocyte and plasma cell infiltrate, scarcity of blood vessels, and proliferation of fibroblasts leading to fibrosis. It is possible that prolonged IL-6 stimulation leads to inappropriate B and T-cell terminal differentiation which supports the lymphoplasmacytic lesion in Dr. Tolosa’s case. The possibility of fibrosis means that early treatment with steroids is important in preventing cumulative damage.