视神经脊髓炎（neuromyelitis optica,NMO）是不同于多发性硬化（multiple sclerosis,MS）的一种中枢神经系统特发性炎性疾病。NMO高度特异性的水通道蛋白4（aquaporin-4,AQP4）抗体的发现,加深了人们对NMO的认识,并扩展了NMO谱系疾病（neuromyelitis optica spectrum disorders,NMOSD）的定义。NMO诊断国际专家组（International Panel for NMO Diagnosis,IPND）对NMOSD诊断标准进行了修订,并达成2015年NMOSD诊断标准国际共识。该诊断标准取消了NMO的个别定义,而将NMO归入NMOSD。同时,根据AQP4抗体表达状态,分为AQP4抗体阳性和AQP4抗体阴性NMOSD。AQP4抗体阳性NMOSD的诊断要求具备6项核心症状之一;AQP4抗体阴性或无法进行AQP4抗体检测的NMOSD的诊断,要求则更为严格,必须有特征性的MRI表现。本文即对2015年NMOSD诊断标准国际共识中的要点进行解读和评论。
Abstract
Neuromyelitis optica (NMO) is an idiopathic inflammatory central nervous system (CNS) syndrome distinct from multiple sclerosis (MS). The discovery of highly specific antiaquaporin-4 antibody as a diagnostic biomarker for NMO has enabled recognition of NMO and expanded the concept of neuromyelitis optica spectrum disorders (NMOSD). The International Panel for NMO Diagnosis (IPND) was convened to develop revised diagnostic criteria of NMOSD—International Consensus Diagnostic Criteria for NMOSD, 2015. In this new nomenclature, the individual definition of NMO is cancelled and classified into unified term NMOSD, which is stratified further by serologic testing (AQP4-immunoglobulin G-positive or -negative). One of the core clinical characteristics is necessarily required for patients with AQP4-immunoglobulin G-positive NMOSD. More stringent clinical criteria, with additional neuroimaging findings, is required for diagnosis of AQP4-immunoglobulin G-negative NMOSD or when serologic testing is unavailable. This paper presents the highlights of International Consensus Diagnostic Criteria for NMOSD, 2015, and gives some comments.
关键词
视神经脊髓炎谱系疾病 /
诊断标准 /
指南 /
水通道蛋白4
Key words
Neuromyelitis optica spectrum disorders /
Diagnostic criteria /
Guideline /
Aquaporin-4
{{custom_sec.title}}
{{custom_sec.title}}
{{custom_sec.content}}
参考文献
[1] WINGERCHUK D M, LENNON V A, LUCCHINETTI C F, et al. The spectrum of neuromyelitis optica[J]. Lancet Neurol, 2007, 6(9):805-815.
[2] LANA-PEIXOTO M A, CALLEGARO D. The expanded spectrum of neuromyelitis optica: evidences for a new definition[J]. Arq Neuropsiquiatr, 2012, 70(10):807-813.
[3] Wingerchuk D M, Banwell B, Bennett J L, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders[J]. Neurology, 2015, 85(2):177-189.
[4] SELLNER J, BOGGILD M, CLANET M, et al. EFNS guidelines on diagnosis and management of neuromyelitis optica[J]. Eur J Neurol, 2010, 17(8):1019-1032.
[5] KIM H J, PAUL F, LANA-PEIXOTO M A, et al. MRI characteristics of neuromyelitis optica spectrum disorder: an international update[J]. Neurology, 2015, 84(11):1165-1173.
[6] Popescu BF, Lennon VA, Parisi JE, et al. Neuromyelitis optica unique area postrema lesions: nausea, vomiting, and pathogenic implications[J]. Neurology, 2011, 76(14):1229-1237.
[7] ZEKERIDOU A, LENNON V A. Aquaporin-4 autoimmunity[J]. Neurol Neuroimmunol Neuroinflamm, 2015, 2(4):e110.
[8] WINGERCHUK D M, WEINSHENKER B G. The emerging relationship between neuromyelitis optica and systemic rheumatologic autoimmune disease[J]. Mult Scler, 2012, 18(1):5-10.
[9] KOWARIK M C, SOLTYS J, BENNETT J L. The treatment of neuromyelitis optica[J]. J Neuroophthalmol, 2014, 34(1):70-82.
[10] TREBST C, JARIUS S, BERTHELE A, et al. Update on the diagnosis and treatment of neuromyelitis optica: recommendations of the Neuromyelitis Optica Study Group (NEMOS)[J]. J Neurol, 2014, 261(1):1-16.
[11] SATO D K, CALLEGARO D, LANA-PEIXOTO M A, et al. Seronegative Neuromyelitis Optica Spectrum—the challenges on disease definition and pathogenesis[J]. Arq Neuropsiquiatr, 2014, 72(6):445-450.
基金
国家自然科学基金项目（编号：81230027）
PDF(1305 KB)
