Case Presentation:

A 41 year old Hispanic female with history of methylenetetrahydrofolate reductase deficiency and migraines presented with worsening right sided retro-orbital headache, new onset diplopia, right facial numbness and right facial droop. She presented to our institution with a severe headache along with new onset diplopia and right facial droop and numbness. Her vital signs on presentation were within normal limits. On physical exam she had complete palsy of right oculomotor, trochlear and abducens nerves, as well as trigeminal nerve and facial nerve impairment. The remainder of her exam was normal. Cranial MRI and MRA with and without contrast revealed abnormal enhancement along right cavernous sinus, extending to right orbital apex, right infratemporal facial nerve and evidence of interval narrowing of right cavernous carotid artery. Extensive work up was performed to exclude infection, inflammation, autoimmune, vascular, endocrine and malignant etiologies. With other likely diagnoses excluded the patient was diagnosed with Tolosa-Hunt syndrome. She was started on Prednisone 100 mg daily for 3 days with an 8-week taper. Follow up visits show complete resolution of headache, improvement in eye movement, with mild residual right abducens, trigeminal and facial nerve paresis. A 6-week follow up MRI brain demonstrates improvement of right cavernous sinus region.

Discussion:

Tolosa-Hunt syndrome is a rare disorder associated with granulomatous inflammation of the cavernous sinus which leads to dysfunction of the structures located within it.  The syndrome is characterized by unilateral painful ophthalmoplegia, paresis of third, fourth and/or sixth cranial nerves. The diagnostic criteria recommended by the International Headache Society include 1) a unilateral headache, 2) granulomatous inflammation of the cavernous sinus, superior orbital fissure or orbit, 3) paresis of the ipsilateral third, fourth and/or sixth cranial nerves, 4) headache that has preceded the oculomotor paresis by at least 2 weeks and is localized around the ipsilateral brow and eye, and 5) symptoms are not better accounted by an alternative diagnosis. The symptoms rapidly improve with glucocorticoid treatment. 

Conclusions:

There is very limited medical literature describing Tolosa-Hunt syndrome available. Most of papers are case reports, which lack evidence-based evidence in treatment regimen. This case provides a detailed clinical and imaging presentation of Tolosa-Hunt syndrome with abnormal MRI findings involving the right cavernous sinus, orbital apex and facial nerve along with resolution of pain and other symptoms and imaging improvement of lesion. There is suggestion of modifying the current diagnostic criteria of Tolosa-Hunt syndrome by International Classification of Headache Disorders version ICHD-II. Given the rarity of Tolosa Hunt syndrome it is important to keep it in the differential when evaluating patients with unrelenting headaches.