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Neuromyelitis optica: a pilot study of clinical presentation and status of serological biomarker AQP4 among patients admitted to a tertiary centre in NCNS, Sudan
BMC Neuroscience ( IF 2.4 ) Pub Date : 2020-02-28 , DOI: 10.1186/s12868-020-0557-x
Etedal Ahmed AbuElbasher Ibrahim 1, 2 , Fatima Gammer 2 , Alsadig Gassoum 2, 3
Affiliation  

Background Neuromyelitis optica (NMO) is a demyelinating disease primarily affecting the optic nerves and spinal cord. It is distinguished from other demyelinating conditions by the presence of AQP4-IgG and serum aquaporin 4 (AQP4), found mainly in the blood–brain barrier. This descriptive study was conducted from January 2015 to June 2018 at the National Center for Neurological Sciences (NCNS) in Khartoum, Sudan. All participants were Sudanese patients diagnosed with NMO. In our study the selection of cases was based on Dean Wingerchuk diagnostic criteria (2006), which states that the diagnosis of NMO should meet two absolute criteria and two supportive criteria. The absolute criteria are myelitis and optic neuritis, whereas supportive criteria include radiological findings obtained from brain and spinal cord MRI. Furthermore, AQP4-IgG levels were measured from cerebrospinal fluid (CSF) and serum using immunofluorescence. Data were collected by a pre-designed questionnaire and analyzed using SPSS version 17. A p value < 0.05 was considered statistically significant. Results A total of 31 patients were enrolled in this study [6 male (19.4%) and 25 female (80.6%)]. The mean age was 38 ± 12.8 years. Motor and visual difficulties were the initial symptoms and occurred in 21 (67.7%) and 10 (32.3%) patients, respectively. Fundoscopy confirmed optic atrophy in 22 (71.0%) patients. The course of the disease revealed one relapse in 21 patients (67.7%). Seropositive AQP4-IgG were seen in 23 patients (79.31%). There was a significant correlation between AQP4 and response to treatment (p ≤ 0.038). The correlation between serum AQP4-IgG, showed that, complete improvement was detected in 2 patients (6.9%) one of them was positive and the other was negative, 20 (69.0%) patients presented with some disability, among them 18 (62.1%) were positive and 2 (6.9%) were negative, while 7 patients showed no improvement (24.1%) 4 out of them were positive (13.8%) and 3 were negative (10.3%). Conclusion At the initial presentation of NMO, longitudinal myelitis was observed more frequently than optic neuritis. More than two third of the patients showed strong seropositivity for serum AQP4. Most seropositive patients showed a good response to treatment but with residual disabilities.

中文翻译:

视神经脊髓炎:苏丹 NCNS 三级中心入院患者的临床表现和血清学生物标志物 AQP4 状态的初步研究

背景视神经脊髓炎(NMO)是一种主要影响视神经和脊髓的脱髓鞘疾病。它与其他脱髓鞘疾病的区别在于 AQP4-IgG 和血清水通道蛋白 4 (AQP4) 的存在,主要存在于血脑屏障中。这项描述性研究于 2015 年 1 月至 2018 年 6 月在苏丹喀土穆的国家神经科学中心 (NCNS) 进行。所有参与者都是被诊断为 NMO 的苏丹患者。在我们的研究中,病例的选择基于 Dean Wingerchuk 诊断标准(2006),该标准指出 NMO 的诊断应满足两个绝对标准和两个支持标准。绝对标准是脊髓炎和视神经炎,而支持标准包括从脑和脊髓 MRI 获得的放射学发现。此外,使用免疫荧光从脑脊液 (CSF) 和血清中测量 AQP4-IgG 水平。通过预先设计的问卷收集数据并使用 SPSS 17 版进行分析。 p 值 < 0.05 被认为具有统计学意义。结果 本研究共纳入31例患者[男性6例(19.4%),女性25例(80.6%)]。平均年龄为 38 ± 12.8 岁。运动和视觉困难是最初的症状,分别发生在 21 (67.7%) 和 10 (32.3%) 名患者中。眼底镜检查证实 22 名 (71.0%) 患者存在视神经萎缩。病程显示 21 名患者(67.7%)复发 1 次。在 23 名患者 (79.31%) 中观察到血清阳性 AQP4-IgG。AQP4 与治疗反应之间存在显着相关性(p ≤ 0.038)。血清 AQP4-IgG 之间的相关性表明,2例(6.9%)完全好转,1例阳性,1例阴性,20例(69.0%)出现残疾,其中阳性18例(62.1%),阴性2例(6.9%) ,而 7 名患者没有改善(24.1%),其中 4 名呈阳性(13.8%),3 名呈阴性(10.3%)。结论 在 NMO 的初始表现中,纵向脊髓炎比视神经炎更常见。超过三分之二的患者显示出血清 AQP4 的强血清阳性。大多数血清阳性患者对治疗反应良好,但仍有残障。7 名患者无改善 (24.1%),其中 4 名呈阳性 (13.8%),3 名呈阴性 (10.3%)。结论 在 NMO 的初始表现中,纵向脊髓炎比视神经炎更常见。超过三分之二的患者显示出血清 AQP4 的强血清阳性。大多数血清阳性患者对治疗反应良好,但仍有残障。7 名患者无改善 (24.1%),其中 4 名呈阳性 (13.8%),3 名呈阴性 (10.3%)。结论 在 NMO 的初始表现中,纵向脊髓炎比视神经炎更常见。超过三分之二的患者显示出血清 AQP4 的强血清阳性。大多数血清阳性患者对治疗反应良好,但仍有残障。
更新日期:2020-02-28
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