Case Presentation: A 42-year-old woman presented with a chief complaint of a 5-day, constant, 10/10, stabbing headache located behind her left eye that began suddenly. Associated symptoms were blurred vision, diplopia, photophobia, lacrimation, left facial numbness, and difficulty opening her left eye. Her medical history included migraine, obesity, asthma, diverticulitis, and osteoarthritis; home medications were Lortab, olanzapine, and rizatriptan which did not relieve her headache. She was afebrile and hemodynamically stable throughout her hospital course. Examination revealed left ptosis and periorbital numbness. She had a dilated pupil slowly reactive to light, diminished vertical gaze, medial deviation, and decreased visual acuity of the left eye. Labs were significant for mild neutrophilia. ACE receptor antibody, ANA multiplex, and ANCA studies were negative. RPR was non-reactive. Blood cultures were negative. CSF studies, including Lyme disease, were unremarkable. Chest x-ray and head CT scan, MRA, and MRV were unrevealing. However, MRI with contrast showed abnormal enhancement on the left lateral wall of the cavernous sinus. Two days of broad-spectrum IV antibiotics were completed without improvement. The patient was switched to steroids and within one day had considerable pain relief. Her negative workup, suggestive imaging, and response to steroids supported the final diagnosis of Tolosa-Hunt Syndrome (THS).
Discussion: The differential for painful ophthalmoplegia is broad and includes traumatic, infectious, neoplastic, vascular, systemic, and inflammatory etiologies, such as THS. Appropriate hematologic tests, CSF studies, and neuroimaging must be completed. THS has been identified as a unique pathology for 65 years and remains a diagnosis of exclusion. The diagnosis of THS has high sensitivity, but low specificity. MRI is reported to be the most sensitive imaging study for THS. Notably, these MRI findings are nonspecific and can mimic lymphoma, meningioma, or sarcoidosis. Pathologically, THS is a nonspecific granulomatous inflammation within the cavernous sinus or superior orbital fissure that can compromise cranial nerves III, IV, VI and V1. This rare, idiopathic, and relapsing syndrome has no known triggers or geographic, gender, or racial predominance. Symptoms typically resolve without treatment within 8 weeks. Residual nerve palsy is a rare complication. Steroids are known to provide rapid resolution within 72 hours. Several case reports recommend a follow-up MRI every one to two months for two years.
Conclusions: Tolosa-Hunt Syndrome should always be included in the broad differential for painful ophthalmoplegia. Diagnostic criteria for THS has low specificity, therefore it is important to maintain a high suspicion for lymphoma, meningioma, and sarcoidosis. Steroids provide rapid symptomatic improvement, but close follow-up is critical. Several case reports argue that each relapse must be approached as a new diagnosis with a full work-up. A clinical framework for the diagnosis, treatment, and follow-up of THS is essential in order to optimize clinical outcomes.