Atypical Tolosa Hunt syndrome with bilateral sclerokeratitis and optic atrophy in Takayasu's arteritis

PURPOSE: We report an interesting case of atypical Tolosa Hunt syndrome with bilateral Sclerokeratitis and optic atrophy in a patient with Takayasu's arteritis. OBSERVATIONS: A 31-year- old lady presented with severe retroorbital pain in the right eye and right sided headache of 2 weeks duration. She had a history of 6th and 7th nerve palsies which improved with oral corticosteroids. Her BCVA was no perception of light in OD and 6/6 in OS. Slitlamp examination showed bilateral old sclerokeratitis and fundus examination showed bilateral optic atrophy. Systemic examination revealed BP of 240/100 mm of hg in the right upper limb with absent pulsations in the left radial, bilateral femoral and dorsalis pedis arteries noted. CT aortic angiography confirmed the diagnosis of Takayasu's arteritis. Symptoms improved with oral corticosteroids and azathioprine, which was followed by a renal stenting procedure. At 1 year followup, she is doing well with no recurrences. CONCLUSIONS AND IMPORTANCE: This case report presents a unique manifestation of atypical Tolosa Hunt syndrome in Takayasu's arteritis. To our knowledge, atypical Tolosa Hunt syndrome in Takayasu'arteritis has not been described in literature.