Tolosa-Hunt syndrome presenting as persistent unilateral headache and painful ophthalmoplegia: A rare case report highlighting diagnostic challenges and therapeutic response

Abstract

Tolosa-Hunt syndrome (THS) is a rare idiopathic granulomatous inflammation of the cavernous sinus, superior orbital fissure, or orbital apex, presenting with painful ophthalmoplegia. The exact etiology remains unclear, but it is believed to be immune-mediated. Early diagnosis is essential, as prompt corticosteroid therapy results in rapid symptom resolution. This case report highlights the clinical presentation, diagnostic workup, and management of a patient with Tolosa-Hunt syndrome, emphasizing the role of neuroimaging in confirming the diagnosis and the efficacy of corticosteroid treatment. A 55-year-old male presented with a persistent right-sided headache for 2.5 months, accompanied by diplopia for 5 days. Neurological and ophthalmic examination revealed right-sided ptosis, restricted extraocular movements, and binocular diplopia. MRI findings demonstrated an asymmetric enlargement of the right cavernous sinus with inflammatory changes extending into the orbital apex and superior orbital fissure, confirming Tolosa-Hunt syndrome. The patient was treated with high-dose corticosteroids, leading to significant clinical improvement. Following corticosteroid therapy, the patient experienced rapid resolution of headache and improvement in ocular motility. Repeat MRI after 6 weeks showed a marked reduction in inflammation. There was no recurrence of symptoms upon gradual steroid tapering. Tolosa-Hunt syndrome should be considered in patients with unilateral headache and painful ophthalmoplegia. MRI plays a crucial role in diagnosis, and corticosteroids remain the mainstay of treatment, leading to excellent outcomes. Early recognition and intervention can prevent long-term complications and disability.