| 시신경척수염의 치료 |
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김수현, 김우준, 김호진 |
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국립암센터 신경과 |
| Current Treatment Approaches for Neuromyelitis Optica |
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Su-Hyun Kim, Woojun Kim, Ho Jin Kim |
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Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea |
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| Abstract |
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"Neuromyelitis optica (NMO; Devic's syndrome) is an idiopathic severe inflammatory disorder of the central nervous system (CNS) that preferentially involves the optic nerves and spinal cord. Several lines of evidence suggest that NMO is a humorally mediated disease distinct from multiple sclerosis(MS). Such evidence includes immunopathologic studies that demonstrate prominent complement activation and immunoglobulin deposition, clinical observations that systemic autoimmune diseases often coexist with NMO and therapeutic plasmapheresis may provide meaningful rescue for severe clinical attacks, and identification of serum autoantibody marker, NMO-IgG. This autoantibody targets aquaporin-4 (AQP4), the most abundant water channel in the CNS. Anti-AQP4 antibody appears highly specific for NMO and has demonstrated that the spectrum of NMO is broader than previously known. In this context, the new concept of NMO spectrum disorders (NMOSD) was recently introduced, in which may represent CNS AQP4 autoimmunity. As NMOSD often causes severe disability, early initiation of effective therapy is very important to prevent attack-related disability. At present, the treatment of NMOSD is not well-established. High dose intravenous corticosteroids are widely employed as the first-line treatment of acute attacks, whereas therapeutic plasmapheresis is applied in the case of corticosteroids failure. Various strategies for the prevention of relapses have been employed in small case series with modest activity, while clinical experience suggests that standard MS immunomodulatory therapies may worsen the course of NMOSD. We will discuss the treatment options for NMOSD and introduce our experience of repeated treatment of rituximab based on the assessment of peripheral circulating memory B cells and rescue therapy using mitoxantrone. Journal of Multiple Sclerosis 2(2):45-51, 2011" |
| Key Words:
Neuromyelitis optica, Treatment |
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