시신경척수염이 동반된 쇼그렌증후군

쇼그렌증후군의 중추신경계 침범 증상으로 횡단척수염이 있으며 다른 뇌의 백질기능 장애 없이 시신경염과 횡단 척수염이 동반된 경우를 시신경척수염이라고 한다. 저자들은 횡단척수염으로 치료한 기왕력이 있으며 양측 하지 근력 저하 및 이상감각을 주소로 내원한 환자에서 쇼그렌 증후군에 동반된 시신경척수염을 진단하고 고용량 스테로이드 치료에 실패 후 혈장분리교환술로 호전된 예를 경험 하였기에 문헌 고찰과 함께 보고하는 바이다. Neuromyelitis optica (NMO) is an idiopathic inflammatory demyelinating disease, characterized by optic neuritis and myelitis. NMO is a very uncommon and serious neurologic manifestation of Sjogren`s syndrome. We report on a 32-year-old female with NMO as central nerve system involvement of Sjogren`s syndrome. She had a transverse myelitis ten years ago and did not have symptoms for a long period of time. She visited the emergency center because of worsening weakness of both limbs. She had an appendectomy three days ago before hospitalization. Cervical spinal magnetic resonance imaging showed increased signal intensity in T2-weighted images from the cervical (C2) to the upper thoracic (T4) spinal cord. As serum NMO-IgG was positive, we diagnosed neuromyelitis optica and treated with high dose steroid, but failed. Therefore, we treated with plasmapheresis and the patient was discharged without any neurological deficits.