A 45 year-old Hispanic-female was admitted from ophthalmology clinic to the internal medicine ward for 3 week history of left-sided blurry vision, diplopia, opthalmoplegia, ptosis, peri-orbital pain, occipital headaches, impaired V1 and V2 facial sensation, and intermittent ear pain. Her medical history was pertinent for rheumatoid arthritis on immunosuppression and poorly controlled type 2 diabetes. The patient was recently treated for an oral fungal infection and sinusitis. Due to her medical history and recent infection, otolaryngology and ophthalmology were immediately consulted to rule out invasive fungal sinusitis, and her immunosuppression was held.
Her labs were notable for mild chronic leukocytosis and hyperglycemia without any evidence of hepatic or renal impairment. Computed Tomography was concerning for right cavernous sinus thrombosis. Subsequent Magnetic Resonance Venography of the brain demonstrated cordlike area of enhancement extending from left orbital apex to the left cavernous sinus with possible slight medial mass effect on the left internal carotid artery. With this new information, the differential diagnosis included malignancy, pseudotumor cerebri, Tolosa-Hunt syndrome, or an infectious process. A lumbar puncture was performed to rule out infectious and malignant etiologies. The opening pressure was normal, and the cerebral spinal fluid studies were negative Lyme disease, Syphilis, bacteria, fungi, or mycobacteria. Serum ACE, ANA, anti-dsDNA, anti-Smith antibody, ANCA, FTA test, and Lyme disease were also normal. Once infection was ruled out, the patient was started on high dose prednisone 80mg daily with near complete resolution of her symptoms within 3 days. The patient was discharged home with a prolonged steroid taper and follow up with Neuro-Ophthalmology.
Discussion:
Tolosa-Hunt syndrome is a rare syndrome of unknown etiology with favorable prognosis. Due to its nonspecific inflammatory nature within the cavernous sinus or superior orbital fissure, the differential diagnosis is vast, including malignancy, infection, and other inflammatory processes. With an estimated annual incidence of one case per million per year, it is a diagnosis of exclusion requiring clinical and radiologic support.
Our patient met the diagnostic criteria defined by the International Headache Society: unilateral headache, inflammation of the cavernous sinus and orbit demonstrated on MRI, ophthalmoplegia, no alternative diagnoses that account for presentation.
The recommended therapy for those that meet the clinical and objective criteria for Tolosa-Hunt syndrome is high dose prednisone 80mg for an initial 3 days followed by a 2 week interval taper. Marked improvement of symptomology is expected within 24 to 72 hours. Close outpatient follow-up with interval MRI is recommended.
Conclusions:
While Tolosa-Hunt syndrome can often self-resolve, it could potentially result in vision loss. Keen evaluation and prompt recognition by the practicing provider is essential to prevent devastating long term sequela.