土耳其队列中的视神经脊髓炎和视神经脊髓炎谱系障碍患者：人口统计学、临床和实验室特征
Neuromyelitis Optica and Neuromyelitis Optica Spectrum Disorder Patients in Turkish Cohort: Demographic, Clinical, and Laboratory Features.
作者信息
Altintas Ayse, Karabudak Rana, Balci Belgin P, Terzi Murat, Soysal Aysun, Saip Sabahattin, Tuncer Kurne Asli, Uygunoglu Ugur, Nalbantoglu Mecbure, Gozubatik Celik Gokcen, Isik Nihal, Celik Yahya, Gokcay Figen, Duman Taskin, Boz Cavit, Yucesan Canan, Mangan Mehmet Serhat, Celebisoy Nese, Diker Sevda, Colpak Isikay Ilksen, Kansu Tulay, Siva Aksel
机构信息
Departments of *Neurology ∥∥Ophthalmology, Istanbul University Cerrahpasa Medical School ‡Department of Neurology, Haseki Training and Education Hospital ∥Department of Neurology, Bakirkoy Training and Education Hospital ¶Department of Neurology, Istanbul Medeniyet University Goztepe Training and Education Hospital, Istanbul †Department of Neurology, Hacettepe University Medical School §§Department of Neurology, Ankara University Medical School, Ankara §Department of Neurology, Ondokuz Mayis University Medical School, Samsun #Department of Neurology, Trakya University Medical School, Edirne **Department of Neurology, Ege University Medical School, Izmir ††Department of Neurology, Mustafa Kemal University Medical School, Hatay ‡‡Department of Neurology, Karadeniz Technical University Medical School, Trabzon, Turkey.
出版信息
Neurologist. 2015 Oct;20(4):61-6. doi: 10.1097/NRL.0000000000000057.
BACKGROUND
Neuromyelitis optica (NMO) is an immune-mediated, chronic relapsing, inflammatory disease characterized by severe attacks of optic neuritis and myelitis.
OBJECTIVE
To determine the demographic, clinical, and laboratory features; antibody status; and treatment modalities of patients with NMO and neuromyelitis optica spectrum disorders in a Turkish cohort from 11 centers.
METHODS
A total of 182 patients were included in this study. Data on age at disease onset, sex, type of attacks, clinical presentation, analysis of cerebrospinal fluid, serum antiaquaporin-4 antibody status, annual progression index, and medical and family histories were collected.
RESULTS
Mean age was 38.43±12.40 years (range, 13 to 75 y), and mean age at disease onset was 31.29±12.40 years (median, 29 y; range, 10 to 74 y). In NMO group, the rate of NMO immunoglobulin (Ig)G positivity was 62.5%. The annual progression index was significantly higher in the longitudinally extending spinal cord lesion. The mean Expanded Disability Status Scale score was higher in the late than early-onset NMO group.
CONCLUSION
Our results revealed a lower rate of NMO IgG positivity, more severe disability in patients with NMO/neuromyelitis optica spectrum disorders presenting with either transverse myelitis or late-onset NMO, and no correlation between disability and NMO IgG status.
背景
视神经脊髓炎（NMO）是一种免疫介导的慢性复发性炎症性疾病，其特征为严重的视神经炎和脊髓炎发作。
目的
确定来自11个中心的土耳其队列中NMO及视神经脊髓炎谱系障碍患者的人口统计学、临床和实验室特征、抗体状态及治疗方式。
方法
本研究共纳入182例患者。收集了疾病发病年龄、性别、发作类型、临床表现、脑脊液分析、血清抗水通道蛋白4抗体状态、年进展指数以及医疗和家族史等数据。
结果
平均年龄为38.43±12.40岁（范围13至75岁），疾病发病的平均年龄为31.29±12.40岁（中位数29岁；范围10至74岁）。在NMO组中，NMO免疫球蛋白（Ig）G阳性率为62.5%。纵向延伸性脊髓病变的年进展指数显著更高。迟发性NMO组的平均扩展残疾状态量表评分高于早发性NMO组。
结论
我们的结果显示，NMO IgG阳性率较低，患有横贯性脊髓炎或迟发性NMO的NMO/视神经脊髓炎谱系障碍患者残疾程度更严重，且残疾与NMO IgG状态之间无相关性。
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