Tolosa-Hunt syndrome as initial presentation of systemic lupus erythematosus

Purpose: Case presentation of newly diagnosed systemic lupus erythematosus (SLE) presenting initially as Tolosa-Hunt syndrome (THS). Study Design: Retrospective clinical case. Method: Case report. Results: A healthy young man developed acute binocular diplopia within 2 days without other neurological deficits. Bilateral 6th cranial nerve palsy was observed with general reduction in the visual field test. Emergent brain magnetic resonance image (MRI) was performed, which revealed severe inflammation in the cavernous sinus, superior orbital fissure, and apex of the orbit. No cavernous thrombosis or intracranial lesion was shown in the MRI. THS was diagnosed and the patient’s CN 6 palsy recovered quickly after corticosteroid treatment. However, severe anaemia was discovered during admission (Hb=6.0), so the patient was evaluated by profound laboratory tests, which revealed SLE. Conclusion: With painful ophthalmoplegia, cavernous sinus syndrome is highly suspected. THS is one of the differential diagnoses for cavernous sinus syndrome. THS is a rare disease, recognized by the National Organization for Rare Disorders, and characterized by inflammatory changes in the cavernous sinus, superior orbital fissure and/or orbital apex under image study. The inflammatory changes are mostly idiopathic, but secondary causes such as sarcoidosis or other autoimmune diseases need to be ruled out. Physicians should be aware of possible underlying conditions, such as immunosuppressed status as in SLE, as the true cause of THS.