Tolosa-Hunt syndrome with pseudotumor cerebri. Report of an unusual case

An 12-old-year girl with Tolosa-Hunt syndrome (THS) complicated with pseudotumor cerebri is presented. She suffered from alternating, recurrent and painful ophthalmoplegia at the age of 8, and bilateral papilledema which did not affect visual acuity was also recognized. THS subsided readily on steroid therapy, and the remission lasted more than 3 years after discontinuing steroid. However, papilledema did not change despite remission of THS, and resulted in optic atrophy with a mildly enlarged scotoma and 10% decrease in visual acuity 6 months after the onset. MRI disclosed slightly contrasted masses in the bilateral enlarged cavernous sinuses and narrowing of the left carotid siphon. Another small mass with partial gadolinium enhancement was revealed adjacent to the left narrowing of the carotid siphon in the optochiasmatic cistern; however, there was no lesion causing intracranial hypertension. The intracavernous MRI findings were considered characteristic of THS, and papilledema seemed to be due to pseudotumor cerebri by exclusion. Since subsequent MRI confirmed no progression of the above findings, the intracavernous and intracisternal masses were suspected to be non-specific inflammatory granulomas associated with THS.