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视神经脊髓炎谱系疾病伴髓鞘少突胶质细胞糖蛋白或水通道蛋白-4 抗体:阿尔及利亚患者的临床和辅助检查特征。

Neuromyelitis optica spectrum disorders with antibodies to myelin oligodendrocyte glycoprotein or aquaporin-4: Clinical and paraclinical characteristics in Algerian patients.

机构信息

Centre hôspitalo-universitaire Nedir Mohamed, Service de Neurologie, Tizi Ouzou, Algeria.

Centre hôspitalo-universitaire Nedir Mohamed, Service de Neurologie, Tizi Ouzou, Algeria.

出版信息

J Neurol Sci. 2017 Oct 15;381:240-244. doi: 10.1016/j.jns.2017.08.3254. Epub 2017 Aug 31.

DOI:10.1016/j.jns.2017.08.3254
PMID:28991690
Abstract

BACKGROUND

Neuromyelitis optica (NMO) is a severe autoimmune inflammatory disorder of the central nervous system. NMO and its abortive forms are referred to as NMO spectrum disorders (NMOSD). NMOSD are mostly associated with antibodies to aquaporin-4 (AQP4-IgG). However, recent studies have demonstrated antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) in a subset of patients. Data on NMOSD in North Africa are sparse.

OBJECTIVE

To describe the frequency of MOG-IgG and AQP4-IgG among patients with optic neuritis (ON) and/or myelitis in Algeria as well as the clinical and paraclinical features associated with these antibodies.

METHODS

Retrospective testing of 42 patients with optic neuritis and/or myelitis treated at the teaching hospital of TiziOuzou for MOG-IgG and AQP4-IgG, and retrospective evaluation of the patients' medical records.

RESULTS

Six of 42 (14.3%) patients were positive for AQP4-IgG and 3/42 (7.1%) were positive for MOG-IgG. No patient was positive for both AQP4-IgG and MOG-IgG. All antibody-positive patients were women. MOG-IgG was associated with severe episodes of ON in all MOG-IgG-positive patients. Steroid treatment was followed by complete remission in two patients. AQP4-IgG was associated with ON and/or longitudinally extensive transverse myelitis (LETM), often with severe onset. While all six of the AQP4-IgG-positive patients met the 2015 IPND criteria for NMOSD, only one of the three MOG-IgG-positive patients did so. Interestingly, clinically silent extensive spinal cord or brain lesions were present in two of the three MOG-IgG-positive patients, and altered visual evoked potentials without clinical evidence of ON were found in three of the six AQP4-IgG-positive patients.

CONCLUSION

MOG-IgG and AQP4-IgG are found in a substantial subset of Algerian patients with ON and/or myelitis, are present predominantly in women, and may be associated with differences in clinical presentation and, possibly, outcome. Only a subset of MOG-IgG positive patients meets the current diagnostic criteria for NMOSD.

摘要

背景

视神经脊髓炎(NMO)是一种严重的中枢神经系统自身免疫性炎症性疾病。NMO 及其顿挫型被称为 NMO 谱疾病(NMOSD)。NMOSD 主要与水通道蛋白 4(AQP4-IgG)抗体相关。然而,最近的研究表明,在一部分患者中存在髓鞘少突胶质细胞糖蛋白(MOG-IgG)抗体。北非的 NMOSD 数据很少。

目的

描述在阿尔及利亚,视神经炎(ON)和/或脊髓炎患者中 MOG-IgG 和 AQP4-IgG 的频率,以及与这些抗体相关的临床和实验室特征。

方法

对在提济乌祖教学医院接受治疗的 42 例 ON 和/或脊髓炎患者进行 MOG-IgG 和 AQP4-IgG 的回顾性检测,并对患者的病历进行回顾性评估。

结果

42 例患者中,6 例(14.3%)AQP4-IgG 阳性,3 例(7.1%)MOG-IgG 阳性。没有患者同时 AQP4-IgG 和 MOG-IgG 阳性。所有抗体阳性患者均为女性。在所有 MOG-IgG 阳性患者中,MOG-IgG 与严重的 ON 发作有关。在两名患者中,类固醇治疗后完全缓解。AQP4-IgG 与 ON 和/或长节段横贯性脊髓炎(LETM)相关,常伴有严重起病。虽然 6 例 AQP4-IgG 阳性患者均符合 2015 年 NMOSD 的 IPND 标准,但仅 3 例 MOG-IgG 阳性患者中的 1 例符合。有趣的是,在 3 例 MOG-IgG 阳性患者中有 2 例存在临床无症状的广泛脊髓或脑内病变,在 6 例 AQP4-IgG 阳性患者中有 3 例存在视觉诱发电位改变而无 ON 的临床证据。

结论

MOG-IgG 和 AQP4-IgG 在相当一部分阿尔及利亚 ON 和/或脊髓炎患者中存在,主要见于女性,且可能与临床表现和(可能)结局的差异有关。只有一部分 MOG-IgG 阳性患者符合 NMOSD 的现行诊断标准。

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