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秘鲁队列中视神经脊髓炎谱系障碍的临床和副临床特征

Clinical and paraclinical profile of neuromyelitis optic spectrum disorder in a peruvian cohort.

作者信息

Castro-Suarez Sheila, Guevara-Silva Erik, Osorio-Marcatinco Victor, Alvarez-Toledo Kelvin, Meza-Vega Maria, Caparó-Zamalloa César

机构信息

CBI en Demencias y Enfermedades Desmielinizantes del Sistema Nervioso, Instituto Nacional de Ciencias Neurológicas, Lima, Peru; Global Brain Health Institute, University of California, San Francisco, CA, United States.

CBI en Demencias y Enfermedades Desmielinizantes del Sistema Nervioso, Instituto Nacional de Ciencias Neurológicas, Lima, Peru.

出版信息

Mult Scler Relat Disord. 2022 Aug;64:103919. doi: 10.1016/j.msard.2022.103919. Epub 2022 May 29.

DOI:10.1016/j.msard.2022.103919
PMID:35691236
Abstract

BACKGROUND

Neuromyelitis Optica Spectrum Disorders (NMOSD) is an autoimmune, inflammatory disorder of the Central Nervous System that typically involves the spinal cord and the optic nerves. Recently, the clinical and radiological spectrum of NMOSD has been increasing in Latin America. In Peru, there have only been a few clinical reports on NMOSD published. For this reason, we aimed to assess the clinical and paraclinical characteristics of patients with NMOSD from a tertiary-level neurological center in Lima-Peru.

METHODS

This is a descriptive study. We assessed medical reports of patients with NMOSD based on the 2015 diagnostic criteria attended in a goverment institute (Instituto Nacional de Ciencias Neurologicas) from Peru between 2013-2019. Those patients who met diagnostic criteria were selected and analyzed. We analyzed continuous data among groups (AQP4-IgG seropositive and AQP4-IgG seronegative/unknow).

RESULTS

We identified 58 clinical records that met the selection criteria and were included in the study. The highest percentage of patients (53%) were born in the north of Peru (from parallel 0°01'48''S - 6°56'38''S). NMOSD were more prevalent in women (86%), the male:female ratio was 1:6, the mean age at diagnosis was 50 years. AQP4-IgG antibodies were tested in (63.8%), 62% of whom were seropositive and 38% seronegative. The frequency of EO-NMO and LO-NMO was 34.8% and 65.2% in AQP4-IgG seropositive patients, respectively. Unknown AQP4-IgG was found 21 patients. In LO-NMOSD group, AQP4-IgG seropositive was found in a higher percentage. Optic neuritis was the first clinical event at 40% . In the patients who presented myelitis as the first clinical event, 18.2% were AQP4-IgG seropositive, while only 4.8% was found in the rest of the patients. 17% had other associated autoimmune diseases and 16% had anti-nuclear antibodies. 79% of patients had low vitamin D-25(OH) levels (<30ng/ml). Orbit MRI showed unilateral optic neuritis in 46.6%. Spinal cord MRIs showed extensive longitudinal myelitis in 52% of patients and the thoracic segment was the most frequently affected (47%).

CONCLUSIONS

In the present study of a peruvian NMOSD cohort, we found a higher frequency of unilateral optic neuritis cases, and a higher percentage of AQP4-IgG seropositive patients among those older than 50.

摘要

背景

视神经脊髓炎谱系障碍(NMOSD)是一种中枢神经系统的自身免疫性炎症性疾病,通常累及脊髓和视神经。最近,拉丁美洲NMOSD的临床和放射学谱系一直在增加。在秘鲁,仅有少数关于NMOSD的临床报告发表。因此,我们旨在评估来自秘鲁利马一家三级神经科中心的NMOSD患者的临床和辅助检查特征。

方法

这是一项描述性研究。我们根据2015年诊断标准评估了2013年至2019年期间在秘鲁一家政府机构(国家神经科学研究所)就诊的NMOSD患者的医疗报告。选择并分析符合诊断标准的患者。我们分析了各组之间的连续数据(水通道蛋白4-IgG血清阳性和水通道蛋白4-IgG血清阴性/未知)。

结果

我们确定了58份符合入选标准并纳入研究的临床记录。患者中出生于秘鲁北部(南纬0°01'48''至6°56'38'')的比例最高(53%)。NMOSD在女性中更为常见(86%),男女比例为1:6,诊断时的平均年龄为50岁。对63.8%的患者进行了水通道蛋白4-IgG抗体检测,其中62%为血清阳性,38%为血清阴性。在水通道蛋白4-IgG血清阳性患者中,视神经脊髓炎谱系疾病(EO-NMO)和长节段横贯性脊髓炎(LO-NMO)的发生率分别为34.8%和65.2%。发现21例患者水通道蛋白4-IgG情况未知。在LO-NMOSD组中,水通道蛋白4-IgG血清阳性的比例更高。视神经炎是首发临床事件的占40%。在以脊髓炎为首发临床事件的患者中,18.2%为水通道蛋白4-IgG血清阳性,而在其他患者中仅为4.8%。17%的患者有其他相关自身免疫性疾病,16%的患者有抗核抗体。79%的患者维生素D-25(OH)水平较低(<30ng/ml)。眼眶MRI显示46.6%的患者有单侧视神经炎。脊髓MRI显示52%的患者有广泛的纵向脊髓炎,胸段最常受累(47%)。

结论

在本项对秘鲁NMOSD队列的研究中,我们发现单侧视神经炎病例的发生率较高,且在50岁以上患者中,水通道蛋白4-IgG血清阳性患者的比例更高。

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