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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

임진아|염미선|임현택|고태성|이신혜
대한소아신경학회 (the Korean Child Neurology Society)|2008.11|대한소아신경학회지 (Journal of the korean child neurology society)|제16권 제2호|pp. 213-221

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참고문헌

키워드

Transverse myelitisOptic neuritisNeuromyelitis opticaDevic`s syndromeNMO-IgG
주제분류신경과학

초록

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.

Abstract

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.

참고문헌 (37)

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