Tolosa-Hunt Syndrome: A Non-Classical Presentation of a Rare Cause of Unilateral Headache and Painful Ophthalmoplegia

Tolosa-Hunt syndrome is a rare condition involving the orbital and retro-orbital space. The typical symptoms are orbital pain, swelling, headache, palsies of the cranial nerves, and sensory loss in the distribution of the trigeminal nerve. Tolosa-Hunt syndrome relapses and remits with episodes separated by months to years. It is a diagnosis of exclusion after ruling out other causes of painful ophthalmoplegia and is treated with high-dose steroids. We present a case of a 43-year-old woman with a history of recurrent headaches and vision disturbances for the past 4 years presenting with worsening left-eye pain that radiated to the back of her head, swelling around the left eye, double vision, and a unilateral left-sided headache. Visual acuity was decreased in the left eye compared to the right and the patient reported left-eye pain when at extreme medial and lateral gaze. Erythrocyte sedimentation rate (ESR) was found to be elevated with all other laboratory testing within normal limits. Computed tomography (CT) scan of the orbits was unremarkable. Ophthalmology excluded other differential diagnoses and she was treated with intravenous methylprednisolone with significant improvement of symptoms. The triad of one or more episodes of unilateral orbital pain, paresis of one or more of the cranial nerves, and granulomas by magnetic resonance imaging (MRI) or biopsy is 95-100% sensitive at diagnosing the syndrome; however, our patient did not present with the “classic” triad. Initial treatment of Tolosa-Hunt is with high-dose steroids which as in our case lead to rapid and significant improvement of symptoms.