Case Presentation: A 47-year-old male with a past medical history of Bell’s palsy, hypertension, end-stage renal disease on hemodialysis, papillary thyroid cancer s/p thyroidectomy, and post-surgical hypothyroidism presented with new-onset left sided ptosis, mydriasis and retro-orbital pain. Approximately, three weeks ago he experienced similar symptoms without corresponding headache that self-resolved. The review of systems was negative for any focal motor limb deficits, numbness or tingling. Neurological examination was significant for left-sided ptosis and impairment of adduction and elevation of the left eye, consistent with an oculomotor nerve palsy. The left pupil was dilated with no direct or consensual light reflex, but there was no sensory or motor loss in the trigeminal nerve distribution. Bloodwork including ESR/CRP, HIV, VZV/CMV and lumbar puncture was negative. Non-contrast MRI brain and orbits was negative for stroke, masses, optic nerve abnormalities, or cavernous sinus thrombosis, however, contrast study was precluded by renal failure. Therefore, due to high suspicion of Tolosa Hunt Syndrome, we initiated prednisone 80mg daily with a slow taper regimen. Subsequent outpatient follow up 4 weeks later demonstrated resolution of the headache and significant improvement of left oculomotor palsy.
Discussion: Tolosa Hunt syndrome, initially described in 1954, is described by granulomatous inflammation of the cavernous sinus and/or superior orbital fissure resulting in cranial nerve dysfunction of individual or all-encompassing structures. The cranial nerves of concern are third, fourth or sixth cranial nerves, V1 and/or V2 divisions of the fifth cranial nerve. Less than 20 cases are published with a reported incidence of one in a million individuals per year. The syndrome presents with periorbital or hemicranial pain associated with complete/partial ophthalmoplegia and unilateral ptosis. While the cause of this syndrome is unknown, it bears a benign course and patients can be reliably reassured. The syndrome has equal gender distribution and impacts individual between 10-80 years of age. Diagnosis preferentially involves Contrast MRI of the brain which will demonstrate enhancement of the inflammatory portions of the cavernous sinus and exclude sinus thrombosis and intraorbital/intraparenchymal tumors. However, biopsy can be an alternative for diagnostic confirmation. Treatment entails high dose glucocorticoids with taper and results rapid resolution of painful symptoms although resolution of cranial neuropathies may be slower.
Conclusions: This case highlights the importance of meticulously excluding dangerous conditions such as cavernous sinus infection or thrombosis in cases of isolated cranial neuropathy. A thorough neurological exam is paramount for the diagnosis of Tolosa Hunt syndrome. Once diagnosed, appropriate reassurance may be given as steroid therapy is often successful in relieving pain and improving neurological symptoms.