Magaña Setty M, Pittock Sean J, Lennon Vanda A, Keegan B Mark, Weinshenker Brian G, Lucchinetti Claudia F
Department of Neurology, Mayo Clinic College of Medicine, 200 First Ave SW, Rochester, MN 55902, USA.
Arch Neurol. 2009 Aug;66(8):964-6. doi: 10.1001/archneurol.2009.152.
The aquaporin-4-specific serum autoantibody neuromyelitis optica (NMO) IgG is a validated biomarker distinguishing NMO spectrum disorders from multiple sclerosis (MS). Because fulminant attacks are more common in NMO spectrum disorders than in MS, some investigators suggest that NMO IgG may be a marker of destructive demyelination rather than a disease-specific biomarker. To our knowledge, this study is the first to compare NMO IgG serostatus among patients with fulminant central nervous system inflammatory demyelinating disease (CNS IDD).
To determine whether NMO IgG distinguishes patients with NMO spectrum disorders from those with other fulminant corticosteroid-refractory CNS IDD.
Descriptive historical cohort.
Neuroimmunology laboratory and neurology practice, Mayo Clinic College of Medicine, Rochester, Minnesota. Patients Serum samples from 74 patients who underwent plasmapheresis between February 24, 1993, and November 22, 2007, for a corticosteroid-refractory CNS IDD were tested for NMO IgG by indirect immunofluorescence assay.
Two blinded observers scored serum samples tested at 1:120 dilution. Clinical data were obtained by medical record review.
Preplasmapheresis serum samples were available from 74 patients (ratio of women to men, 2:5); the mean interval between blood draw and plasmapheresis was 13 days. At the time of plasmapheresis, the mean age of patients was 46 years (age range, 7-80 years); the mean Expanded Disability Status Scale score was 7.0 (score range, 3.5-9.5 [10.0 is death]). Diagnoses included MS (18 patients with definite and 11 patients with probable), longitudinally extensive transverse myelitis involving at least 3 vertebral segments (20 patients), NMO (14 patients), transverse myelitis involving fewer than 3 vertebral segments (8 patients), optic neuritis (2 patients), and acute disseminated encephalomyelitis (1 patient). Neuromyelitis optica IgG was detected in 20 patients (27%) (10 with longitudinally extensive transverse myelitis, 9 with NMO, and 1 with recurrent optic neuritis) and was not detected in any patient with MS, short transverse myelitis, monophasic optic neuritis, or acute disseminated encephalomyelitis.
Neuromyelitis optica IgG is a specific biomarker for NMO spectrum disorders and is not simply a marker of destructive CNS IDD.
水通道蛋白4特异性血清自身抗体视神经脊髓炎（NMO）IgG是一种经过验证的生物标志物，可用于区分视神经脊髓炎谱系疾病与多发性硬化症（MS）。由于暴发性发作在视神经脊髓炎谱系疾病中比在多发性硬化症中更常见，一些研究人员认为NMO IgG可能是破坏性脱髓鞘的标志物，而不是疾病特异性生物标志物。据我们所知，本研究是首次比较暴发性中枢神经系统炎性脱髓鞘疾病（CNS IDD）患者中NMO IgG血清状态。
确定NMO IgG是否能区分视神经脊髓炎谱系疾病患者与其他暴发性皮质类固醇难治性CNS IDD患者。
描述性历史队列研究。
明尼苏达州罗切斯特市梅奥诊所医学院神经免疫学实验室和神经病学诊所。患者：对1993年2月24日至2007年11月22日期间因皮质类固醇难治性CNS IDD接受血浆置换的74例患者的血清样本进行间接免疫荧光法检测NMO IgG。
两名盲法观察者对1:120稀释度检测的血清样本进行评分。通过病历审查获取临床数据。
74例患者有血浆置换前血清样本（女性与男性比例为2:5）；采血与血浆置换之间的平均间隔为13天。在进行血浆置换时，患者的平均年龄为46岁（年龄范围7 - 80岁）；平均扩展残疾状态量表评分为7.0（评分范围3.5 - 9.5 [10.0为死亡]）。诊断包括MS（明确诊断18例，可能诊断11例）、累及至少3个椎体节段的纵向广泛横贯性脊髓炎（20例）、NMO（14例）、累及少于个椎体节段的横贯性脊髓炎（8例）、视神经炎（2例）和急性播散性脑脊髓炎（1例）。在20例患者（27%）中检测到视神经脊髓炎IgG（10例纵向广泛横贯性脊髓炎、9例NMO和1例复发性视神经炎），而在任何MS、短程横贯性脊髓炎、单相视神经炎或急性播散性脑脊髓炎患者中均未检测到。
视神经脊髓炎IgG是视神经脊髓炎谱系疾病的特异性生物标志物，并非简单的破坏性CNS IDD标志物。
