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视神经脊髓炎谱系疾病脊髓受累患者的残疾、抗水通道蛋白 4 抗体状态与预后的相关性。

Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD.

机构信息

Department of Neurology, New Taipei Municipal TuCheng Hospital, Chang Gung Memorial Hospital and Chang Gung University, New Taipei City, Taiwan.

Department of Neurology, Chang Gung Memorial Hospital Linkou Medical Center and College of Medicine, Chang-Gung University, Linkou, Taoyuan, Taiwan.

出版信息

BMC Neurol. 2021 Apr 9;21(1):153. doi: 10.1186/s12883-021-02171-2.

Abstract

BACKGROUND

Neuromyelitis optica spectrum disorder (NMOSD) is a rare neuroinflammatory disorder of the central nervous system that typically involves the optic nerve, the spinal cord and other specific brain regions. In relapse of the disease, factors associated with clinical features and lesion severity are important for clinicians to predict disease-related disability.

METHODS

We retrospectively analyzed 22 female patients with NMOSD who had spinal cord lesions. Detailed clinical features, onset symptoms, motor disability, relapse episodes, serum aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) autoantibodies and MRI characteristics were documented to correlate their associations with the nadir and three-month Expanded Disability Status Scale (EDSS) scores. Patients with three-month EDSS scores below four (< 4) were categorized as the good outcome group, while those with scores of four or more (> 4) were categorized as the poor outcome group.

RESULTS

In patients with NMOSD, the mean age was 44.5 ± 12.8 years, and the mean three-month EDSS score was 4.3 ± 1.9. A significantly higher all-limb muscle power score was found in the good EDSS group than in the poor EDSS group (p = 0.01). A tendency toward longer follow-up periods and lower anti-AQP4 antibody levels was found in the good outcome group. Serum anti-AQP4 antibodies were present in 86% of patients with NMOSD, and MOG autoantibodies were found in one anti-AQP4 antibody-negative patient (33.3%). In patients with NMOSD, more than 40% of spinal cord lesions were distributed at the middle cervical and upper thoracic levels.

CONCLUSIONS

Our findings suggest that EDSS scores and MRC scores at the nadir had significant associations with three-month EDSS scores. The topographic distributions of the spinal cord lesions might relate to different serum anti-AQP4 antibody status. However, further studies will be needed to corroborate this finding.

摘要

背景

视神经脊髓炎谱系疾病(NMOSD)是一种罕见的中枢神经系统神经炎症性疾病,通常累及视神经、脊髓和其他特定的脑区。在疾病复发时,与临床特征和病变严重程度相关的因素对临床医生预测与疾病相关的残疾很重要。

方法

我们回顾性分析了 22 例女性 NMOSD 脊髓病变患者。详细记录了临床特征、首发症状、运动障碍、复发次数、血清水通道蛋白-4(AQP4)和髓鞘少突胶质细胞糖蛋白(MOG)自身抗体及 MRI 特征,以分析其与疾病早期和三个月扩展残疾状况量表(EDSS)评分的相关性。将三个月 EDSS 评分低于 4(<4)的患者分为预后良好组,评分 4 或更高(>4)的患者分为预后不良组。

结果

NMOSD 患者的平均年龄为 44.5±12.8 岁,平均三个月 EDSS 评分为 4.3±1.9。预后良好组的全肢肌力评分明显高于预后不良组(p=0.01)。预后良好组的随访时间较长,抗 AQP4 抗体水平较低。NMOSD 患者中 86%存在血清抗 AQP4 抗体,1 例抗 AQP4 抗体阴性患者(33.3%)存在 MOG 自身抗体。NMOSD 患者中超过 40%的脊髓病变分布在颈中段和胸上段。

结论

我们的研究结果表明,疾病早期的 EDSS 评分和 MRC 评分与三个月的 EDSS 评分有显著相关性。脊髓病变的分布可能与不同的血清抗 AQP4 抗体状态有关。然而,还需要进一步的研究来证实这一发现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e575/8033738/42192d21c713/12883_2021_2171_Fig1_HTML.jpg

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