Man with Right Eye Pain and Double Vision

Abstract

A 39-year-old previously healthy male presented with three days of right eye pressure and one day of binocular diplopia. He denied history of trauma, headache, or other neurological complaints. He had normal visual acuity, normal intraocular pressure, intact convergence, and no afferent pupillary defect. His neurologic examination was non-focal except for an inability to adduct the right eye past midline on cranial nerve examination, with left-beating nystagmus noted in the left eye. MRI brain was obtained, showing right cavernous sinus lateral wall contrast enhancement. No other evidence of neoplasm, ischemia, infection, or aneurysm was found. The patient’s deficits rapidly improved with corticosteroids. This is the hallmark of Tolosa-Hunt syndrome (THS), characterized as idiopathic inflammation of the cavernous sinus. The patient was discharged on a prednisone taper, with continued improvement at outpatient neurology follow-up. Tolosa-Hunt syndrome is one of the rare disorders recognized by the National Organization for Rare Disorders (NORD), and has an estimated incidence of one case per million per year. Topics Tolosa-Hunt syndrome, cranial nerve deficits, diplopia, neurologic examination.

One in a Million: A Woman Presenting with Unilateral Painful Ophthalmoplegia

“… 3 Granulomatous inflammation involving lymphocytes and plasma cells increases the pressure within the cavernous sinus and may result in compression of cranial nerves III, IV, and VI as well as the sympathetic plexus surrounding the internal carotid artery. 4 , 5 While the etiology is presumed to be idiopathic, there is a strong association with autoimmune disorders such as systemic lupus erythematosus and sarcoidosis. 6 , 7 Magnetic resonance imaging studies of the cavernous sinus and petrous apex are highly sensitive for THS and characteristically show enlargement and focal-enhancing masses within the affected cavernous sinus.…”