To describe a case of complete painful ophthalmoplegia and the broad work-up that goes into the exclusionary diagnosis of Tolosa-Hunt Syndrome.

Based on the International Classification of Headache Disorders, Tolosa-Hunt Syndrome is a unilateral orbital/periorbital pain associated with paresis of one or more of the 3^rd, 4^th, and/or 6^th cranial nerves caused by granulomatous inflammation in the cavernous sinus, superior orbital fissure, or orbit. It is a diagnosis of exclusion and is a rare cavernous sinus syndrome that accounts for <5% of painful ophthalmoplegia.  

A 54-year-old, left-handed male presented to several institutions with episodic right-sided orbital/periorbital headache and diplopia found to have complete right eye ophthalmoplegia, diminished sensation to his right forehead, possible decreased right corneal sensation, and mild right proptosis on neurological examination. Radiographically, he was also found to have slight narrowing of the right ICA as it transitions from the petrous canal into the cavernous sinus, opacification of the right ophthalmic artery, and enhancement along the lateral right cavernous sinus and orbital apex. Additionally, he also had a rare ANA pattern associated with systemic sclerosis, Raynaud’s phenomenon, and some malignancies, as well as a 1-year history of significant pruritic skin lesions involving the bilateral lower extremities attributed to occupational exposure while working with granite.  The patient had a good clinical response to empiric prednisone, including complete resolution of his headache, and was discharged home on a steroid taper. 

This case highlights the importance of understanding the critical structures housed in the cavernous sinus and their clinical relevance as it pertains to the evaluation of painful ophthalmoplegia. Particularly, as the diagnosis of the eponymous Tolosa-Hunt Syndrome is a diagnosis of exclusion that requires a broad differential and extensive work-up.