급성 양측성 중심 망막 동맥 폐쇄를 동반한 시신경 척수염 1례

A Case of Neuromyelitis Optica(Devic's Syndrome) with Acute Bilateral Central Retinal Artery Occlusion

시신경 척수염은 일측성 또는 양측성 시신경염과 횡단 척수염이 동시에 또는 시간 간격을 두고 발생하는 원인미상의 탈수초성 질환이다. 소아에서는 매우 드물며, 다른 여러 탈수초성 질환들과 임상 양상만으로는 감별이 어려운 경우가 많다. 저자들은 양측 망막 동맥 폐쇄를 동반한 시신경염 소견과 지연되어 나타난 하지마비를 보인 8세 여아에서 뇌척수 자기공명영상, 뇌척수액 검사, 혈청 NMO-IgG 검사를 통해 시신경 척수염을 진단하고 유지요법으로 rituximab을 사용하여 이를 보고하는 바이다.

Neuromyelitis optica(NMO) or Devic's syndrome is an uncommon clinical syndrome associating with unilateral or bilateral optic neuritis and transverse myelitis. It is rarely found in children and usually reported in adults with serious neurologic manifestations. We report a case of an 8-year-old girl with neuromyelitis optica whose first clinical manifestation was acute visual loss of both eyes. Initially the patient had been diagnosed with central retinal artery occlusion and optic neuritis by ophthalmologic examination, a brain magnetic resonance imaging, and cerebrospinal fluid findings. She was treated with intravenous methylprednisolone pulse therapy and heparinization. Then the treatments were replaced with oral prednisolone and warfarin. At the fifteenth day after the start of oral prednisolone tapering, she visited our emergency room for voiding difficulty and paresthesia on both legs. A spinal magnetic resonance imaging revealed increased signal intensity in T2-weighted images from cervical to lumbar level, and neuromyelitis optica- IgG(NMO-IgG) was detected in the patient's serum. After we diagnosed her as having neuromyelitis optica, intravenous methylprednisolone and nine courses of daily plasmapheresis were tried. However, the patient still had visual loss, pain, and sensory loss below the sixth thoracic dermatome, and we tried maintenance therapy with intravenous rituximab. We report our case with reviews of the related literatures.