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Clinical features, gender differences, disease course, and outcome in neuromyelitis optica spectrum disorder
Annals of Indian Academy of Neurology ( IF 1.7 ) Pub Date : 2021-03-01 , DOI: 10.4103/aian.aian_334_20 Nishita Singh 1 , Rohit Bhatia 1 , Prerna Bali 1 , V Sreenivas 2 , M V Padma 1 , Vinay Goyal 1 , Rohit Saxena 3 , Deepa Dash 1 , Ajay Garg 4 , S Leve Joseph 4
Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is an astrocytopathy with a predilection for the optic nerve, spinal cord, and brainstem. In this ambispective study, we evaluate clinical characteristics, responses to therapy, and disability outcomes in patients with NMOSD. Methods: Patients diagnosed as NMOSD and following up for at least 1 year at a tertiary care center in India were recruited. Patient data were collected ambispectively from January 2012 until December 2018. Results: A total of 106 patients (29M/77F) with NMOSD were evaluated. The mean age of onset was 29 (±11.6) years. About 77 patients (72.64%) were positive for the AQP4 antibody. Age of onset was higher for those presenting with an opticospinal syndrome (34.2 years) as compared to either isolated longitudinally extensive transverse myelitis (LETM) (30 years) or optic neuritis (ON) (25.3 years). The most common syndrome at onset was LETM in 57 patients (53.77%) followed by ON in 31 patients (29.24%). Azathioprine was the most common immunotherapy (83.96%) prescribed followed by rituximab (7.54%) and mycophenolate mofetil (1.88%). There was a significant decrease in the number of relapses post-azathioprine (P < 0.001). Out of 67 patients with ON, 21 (31.34%) had complete recovery while 17 (25.37%) patients had a severe deficit at a 3-month follow-up. Out of 92 patients with a motor deficit, 49 (53.26%) patients had a partial motor deficit at a 6-month follow-up. The severe visual deficit at baseline and female gender predicted poor visual and motor recovery, respectively. Conclusion: This is the largest descriptive study on patients with NMOSD from India. Relapse rates were similar irrespective of the clinical presentation, age, gender, and disease course. Treatment with immunosuppressive treatment significantly affected the disease course.
中文翻译：
视神经脊髓炎谱系障碍的临床特征、性别差异、病程和结局
简介：视神经脊髓炎谱系障碍 (NMOSD) 是一种星形细胞病，好发于视神经、脊髓和脑干。在这项具有前瞻性的研究中，我们评估了 NMOSD 患者的临床特征、对治疗的反应和残疾结果。方法：招募被诊断为 NMOSD 并在印度三级护理中心随访至少 1 年的患者。从 2012 年 1 月到 2018 年 12 月，患者数据是双向收集的。结果：共评估了 106 名 NMOSD 患者 (29M/77F)。平均发病年龄为 29 (±11.6) 岁。约 77 名患者 (72.64%) AQP4 抗体呈阳性。与孤立的纵向广泛性横贯性脊髓炎（LETM）（30 岁）或视神经炎（ON）（25.3 岁）相比，出现视脊髓综合征的患者的发病年龄（34.2 岁）更高。发病时最常见的综合征是 57 名患者 (53.77%) 的 LETM，其次是 31 名患者 (29.24%) 的 ON。硫唑嘌呤是最常见的免疫疗法（83.96%），其次是利妥昔单抗（7.54%）和霉酚酸酯（1.88%）。硫唑嘌呤后复发次数显着减少（P< 0.001)。在 67 名 ON 患者中，21 名（31.34%）患者完全康复，而 17 名（25.37%）患者在 3 个月的随访中出现严重缺陷。在 92 名运动障碍患者中，49 名（53.26%）患者在 6 个月的随访中出现部分运动障碍。基线和女性的严重视力缺陷分别预示着视力和运动恢复不佳。结论：这是对印度 NMOSD 患者的最大描述性研究。无论临床表现、年龄、性别和病程如何，复发率相似。免疫抑制治疗显着影响病程。
更新日期：2021-04-29
Annals of Indian Academy of Neurology ( IF 1.7 ) Pub Date : 2021-03-01 , DOI: 10.4103/aian.aian_334_20 Nishita Singh 1 , Rohit Bhatia 1 , Prerna Bali 1 , V Sreenivas 2 , M V Padma 1 , Vinay Goyal 1 , Rohit Saxena 3 , Deepa Dash 1 , Ajay Garg 4 , S Leve Joseph 4
Affiliation
Introduction: Neuromyelitis optica spectrum disorder (NMOSD) is an astrocytopathy with a predilection for the optic nerve, spinal cord, and brainstem. In this ambispective study, we evaluate clinical characteristics, responses to therapy, and disability outcomes in patients with NMOSD. Methods: Patients diagnosed as NMOSD and following up for at least 1 year at a tertiary care center in India were recruited. Patient data were collected ambispectively from January 2012 until December 2018. Results: A total of 106 patients (29M/77F) with NMOSD were evaluated. The mean age of onset was 29 (±11.6) years. About 77 patients (72.64%) were positive for the AQP4 antibody. Age of onset was higher for those presenting with an opticospinal syndrome (34.2 years) as compared to either isolated longitudinally extensive transverse myelitis (LETM) (30 years) or optic neuritis (ON) (25.3 years). The most common syndrome at onset was LETM in 57 patients (53.77%) followed by ON in 31 patients (29.24%). Azathioprine was the most common immunotherapy (83.96%) prescribed followed by rituximab (7.54%) and mycophenolate mofetil (1.88%). There was a significant decrease in the number of relapses post-azathioprine (P < 0.001). Out of 67 patients with ON, 21 (31.34%) had complete recovery while 17 (25.37%) patients had a severe deficit at a 3-month follow-up. Out of 92 patients with a motor deficit, 49 (53.26%) patients had a partial motor deficit at a 6-month follow-up. The severe visual deficit at baseline and female gender predicted poor visual and motor recovery, respectively. Conclusion: This is the largest descriptive study on patients with NMOSD from India. Relapse rates were similar irrespective of the clinical presentation, age, gender, and disease course. Treatment with immunosuppressive treatment significantly affected the disease course.
中文翻译：
视神经脊髓炎谱系障碍的临床特征、性别差异、病程和结局
简介：视神经脊髓炎谱系障碍 (NMOSD) 是一种星形细胞病，好发于视神经、脊髓和脑干。在这项具有前瞻性的研究中，我们评估了 NMOSD 患者的临床特征、对治疗的反应和残疾结果。方法：招募被诊断为 NMOSD 并在印度三级护理中心随访至少 1 年的患者。从 2012 年 1 月到 2018 年 12 月，患者数据是双向收集的。结果：共评估了 106 名 NMOSD 患者 (29M/77F)。平均发病年龄为 29 (±11.6) 岁。约 77 名患者 (72.64%) AQP4 抗体呈阳性。与孤立的纵向广泛性横贯性脊髓炎（LETM）（30 岁）或视神经炎（ON）（25.3 岁）相比，出现视脊髓综合征的患者的发病年龄（34.2 岁）更高。发病时最常见的综合征是 57 名患者 (53.77%) 的 LETM，其次是 31 名患者 (29.24%) 的 ON。硫唑嘌呤是最常见的免疫疗法（83.96%），其次是利妥昔单抗（7.54%）和霉酚酸酯（1.88%）。硫唑嘌呤后复发次数显着减少（P< 0.001)。在 67 名 ON 患者中，21 名（31.34%）患者完全康复，而 17 名（25.37%）患者在 3 个月的随访中出现严重缺陷。在 92 名运动障碍患者中，49 名（53.26%）患者在 6 个月的随访中出现部分运动障碍。基线和女性的严重视力缺陷分别预示着视力和运动恢复不佳。结论：这是对印度 NMOSD 患者的最大描述性研究。无论临床表现、年龄、性别和病程如何，复发率相似。免疫抑制治疗显着影响病程。
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