to report a rare case of exophthalmos with painful ophthalmoplegia revealing Tolosa Hunt syndrome in a young patient with no previous medical history.
Department of Ophthalmology, Habib Bourguiba University Hospital, Faculty of Medicine, University of Sfax, Tunisia.
A 26-year-old woman presented with painful eye movements and diplopia. She has not previous medical history. Examination revealed left ophthalmoplegia, ptosis with exophthalmos and equally reactive pupils. Visual acuity was 7/10 in the left eye, 10/10 in the right eye. There was no relative afferent pupillary defect. Fundoscopic examination was normal in both eyes. The remainder of the neurologic examination was unremarkable. Brain magnetic resonance imaging revealed left orbital apexitis with diffusely enhancing left orbital apex extending to the anterior cavernous sinus and oculomotor muscles. The immunological and infectious assessments were negative. The diagnosis of Tolosa-Hunt syndrome was made, and the patient received corticosteroids. Outcome was favorable with complete regression of oculomotor disorders, exophthalmos and other symptoms after few days of treatment.
Tolosa–Hunt syndrome is an inflammatory process adjacent to the cavernous sinus or within the superior orbital fissure and/or orbital apex. It is a rare entity well-defined clinically but of unknown etiopathogenesis. The clinical diagnostic criteria of this disease include an episode or episodes of unilateral orbital pain; paralysis of one or more of the third, fourth, and sixth cranial nerves; and resolution of symptoms within 72 hours after corticosteroid therapy, with exclusion of other causes. Magnetic resonance imaging findings show inflammatory changes within anterior cavernous sinus, with or without involvement of the internal carotid artery, superior orbital fissure, orbital apex, and/or optic nerve. Although it is often a benign condition, it may lead to optic nerve involvement and blindness. Therefore, it is important to recognize the clinical features of this disease and provide immediate treatment to preserve visual and ocular motility function. In our case, the negativity of investigations and spectacular response to corticosteroids allowed us to retain the diagnosis of Tolosa-Hunt syndrome.
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