中国神经再生研究(英文版)

中国神经再生研究(英文版) ›› 2016, Vol. 11 ›› Issue (10): 1633-1637.doi: 10.4103/1673-5374.193243

• 原著:退行性病与再生 • 上一篇    下一篇

伴/不伴有原发性干燥综合征的视神经脊髓炎谱系疾病患者：谁的脑部异常比例高？

  

  • 收稿日期:2016-09-12 出版日期:2016-10-31 发布日期:2016-10-31

Higher frequency of brain abnormalities in neuromyelitis optica spectrum disorder patients without primary Sj?gren’s syndrome

Li-na Gu1, #, Min Zhang2, #, Hui Zhu3, Jing-yao Liu3, *   

  1. 1 Department of Intensive Care Unit, First Hospital, Jilin University, Changchun, Jilin Province, China 2 Department of Acupuncture and Moxibustion, Changchun University of Chinese Medicine, Changchun, Jilin Province, China 3 Department of Neurology, First Hospital, Jilin University, Changchun, Jilin Province, China
  • Received:2016-09-12 Online:2016-10-31 Published:2016-10-31
  • Contact: Jing-yao Liu, Ph.D., xiaohua208@sina.com.

PDF

522

摘要：

视神经脊髓炎谱系疾病可与原发性干燥综合征同时存在。作者通过对6例伴有原发性干燥综合征的视神经脊髓炎谱系疾病患者及10例不伴有原发性干燥综合征的视神经脊髓炎谱系疾病的临床表现、实验室检查及影像学特点进行对比发现，2组患者的人口学和首发症状情况接近，而伴有原发性干燥综合征的视神经脊髓炎谱系疾病患者的C-反应蛋白水平以及抗核抗体、抗干燥综合征A抗体和抗干燥综合征B抗体阳性者比例高于不伴有原发性干燥综合征的患者。67%(4/6)伴有原发性干燥综合征的视神经脊髓炎谱系疾病的患者及60%(6/10)不伴有原发性干燥综合征的视神经脊髓炎谱系疾病的患者检测出抗水通道蛋白4抗体。不伴有原发性干燥综合征的视神经脊髓炎谱系疾病的患者存在大脑畸形的比例高于伴有原发性干燥综合征的患者。50%(5/10)不伴有原发性干燥综合征的视神经脊髓炎谱系疾病的患者及67%(4/6)伴有原发性干燥综合征的视神经脊髓炎谱系疾病的患者存在超过3个节段的脊髓病变。结果说明伴有和不伴有原发性干燥综合征的视神经脊髓炎谱系疾病患者的临床特征接近，仅从临床表现上难以区别这2组疾病，但不伴有原发性干燥综合征的视神经脊髓炎谱系疾病患者脑部异常比例较高。 

orcid: 0000-0002-9999-7546 (Jing-yao Liu)

关键词: 神经再生, 视神经脊髓炎, 原发性干燥综合征, 视神经脊髓炎谱系疾病, 口干症, 干眼症, 神经损害, MRI, 抗水通道蛋白4

Abstract: Neuromyelitis optica spectrum disorder ofen co-exists with primary Sj?gren’s syndrome. We compared the clinical features of 16 neuromyelitis optica spectrum disorder patients with (n = 6) or without primary Sj?gren’s syndrome (n = 10). All patients underwent extensive clinical, laboratory, and MRI evaluations. Tere were no statistical di?erences in demographics or frst neurological involvement at onset between neuromyelitis optica spectrum disorder patients with and without primary Sj?gren’s syndrome. The laboratory findings of cerebrospinal ?uid oligoclonal banding, serum C-reactive protein, antinuclear autoantibody, anti-Sj?gren’s-syndrome-related antigen A antibodies, anti-Sj?gren’s-syndrome-related antigen B antibodies, and anti-Sm antibodies were signifcantly higher in patients with primary Sj? gren’s syndrome than those without. Anti-aquaporin 4 antibodies were detectable in 67% (4/6) of patients with primary Sj?gren’s syndrome and in 60% (6/10) of patients without primary Sj?gren’s syndrome. More brain abnormalities were observed in patients without primary Sj?gren’s syndrome than in those with primary Sj?gren’s syndrome. Segments lesions (> 3 centrum) were noted in 50% (5/10) of patients without primary Sj?gren’s syndrome and in 67% (4/6) of patients with primary Sj?gren’s syndrome. Tese fndings indicate that the clinical characteristics of neuromyelitis optica spectrum disorder patients with and without primary Sj?gren’s syndrome are similar. However, neuromyelitis optica spectrum disorder patients without primary Sj?gren’s syndrome have a high frequency of brain abnormalities.

Key words: nerve regeneration, neuromyelitis optica, primary Sj?gren’s syndrome, neuromyelitis optica spectrum disorder, xerostomia, xerophthalmia, neurological involvements, magnetic resonance imaging, anti-aquaporin 4, neural regeneration