A Case of Unusual Tolosa-Hunt Syndrome

비전형 Tolosa-Hunt Syndrome 1예

The Tolosa-Hunt syndrome is one of the rare disease with facial, especially peri-orbital pain, and ophthalomoplegia associated mostly with granulomatous lesions in cavernous sinus or superior orbital fissure. In addition to ophthalmoplegia by multiple cranial nerve involvement, the sympathetic nervous system may also be involved leading to Horner's syndrome. A typical Tolosa-Hunt syndrome has a neuro-radiologic finding of an increased density in the involved region, and a laboratory finding of an elevated ESR, as well as a dramatic response to systemic corticosteroid therapy. An unusual case of the Tolosa-Hunt syndrome with normal radiologic and laboratory findings, unresponsive to systemic corticosteroid, and some response of pain relief to a stellate ganglion block, is presented.

우측 암면부, 특히 안구주위에 심한 통증을 호소하고 안검하수와 더불어 우측안구의 완전마비를 동반한 선천성 난장이 환자를 경험하였던 바 이는 신경방사선과학적소견, 검사실 검사소견, 전신적 corticosteroid치료에 대한 반응이 전형적인 Tolosa-Hunt Syndrome과는 다른 비전형적인 Tolosa-Hunt Syndrome이었기에 문헌적 고찰과 함께 보고하는 바이다.