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中国中部首发髓鞘少突胶质细胞糖蛋白抗体病患儿和成人的临床和放射学特征及发病严重程度预测因素分析。

Clinical and Radiological Characteristics of Children and Adults With First-Attack Myelin Oligodendrocyte Glycoprotein Antibody Disease and Analysis of Risk Factors for Predicting the Severity at Disease Onset in Central China.

机构信息

Department of Neurology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.

出版信息

Front Immunol. 2021 Dec 15;12:752557. doi: 10.3389/fimmu.2021.752557. eCollection 2021.

Abstract

OBJECTIVE

To analyze and compare different clinical, laboratory, and magnetic resonance imaging characteristics between pediatric and adult patients with first-attack myelin oligodendrocyte glycoprotein antibody disease (MOGAD) and to explore predictive factors for severity at disease onset.

METHODS

Patients diagnosed with MOGAD at the First Affiliated Hospital of Zhengzhou University from January 2013 to August 2021 were enrolled in this retrospective study. Age at disease onset, sex, comorbidities, laboratory tests, magnetic resonance imaging (MRI) characteristics, and Expanded Disability Status Scale (EDSS) scores were collected and analyzed. The association between risk factors and initial EDSS scores at disease onset was analyzed using logistic regression models and Spearman correlation analyses. A receiver-operating characteristic (ROC) curve analysis was used to evaluate the predictive ability of the uric acid and homocysteine (Hcy) levels for the severity of neurological dysfunction at the onset of MOGAD.

RESULTS

Sixty-seven patients (female, n=34; male, n=33) with first-attack MOGAD were included in this study. The mean age at onset was 26.43 ± 18.22 years (range: 3-79 years). Among patients <18 years of age, the most common presenting symptoms were loss of vision (36.0%), and nausea and vomiting (24.0%), and the most common disease spectrum was acute disseminated encephalomyelitis (ADEM) (40.0%). Among patients aged ≥18 years, the most common presenting symptoms were loss of vision (35.7%), paresthesia (33.3%), and paralysis (26.2%), and the most common disease spectrum was optic neuritis (35.7%). The most common lesions were cortical gray matter/paracortical white matter lesions in both pediatric and adult patients. Uric acid [odds ratio (OR)=1.014; 95% confidence interval (CI)=1.006-1.022; P=0.000] and serum Hcy (OR=1.125; 95% CI=1.017-1.246; P=0.023) levels were significantly associated with the severity of neurological dysfunction at disease onset. Uric acid levels (r=0.2583; P=0.035) and Hcy levels (r=0.3971; P=0.0009) were positively correlated with initial EDSS scores. The areas under the ROC curve were 0.7775 (95% CI= 0.6617‒0.8933; P<0.001) and 0.6767 (95% CI=0.5433‒0.8102, P=0.014) for uric acid and Hcy levels, respectively.

CONCLUSION

The clinical phenotype of MOGAD varies in patients of different ages. The most common disease spectrum was ADEM in patients aged<18 years, while optic neuritis was commonly found in patients aged ≥18 years. The uric acid and Hcy levels are risk factors for the severity of neurological dysfunction at disease onset in patients with first-attack MOGAD.

摘要

目的

分析和比较首发髓鞘少突胶质细胞糖蛋白抗体病(MOGAD)患儿和成人患者的临床、实验室和磁共振成像特征,并探讨发病时严重程度的预测因素。

方法

本回顾性研究纳入了 2013 年 1 月至 2021 年 8 月在郑州大学第一附属医院确诊为 MOGAD 的患者。收集并分析了年龄、性别、合并症、实验室检查、磁共振成像(MRI)特征和扩展残疾状况量表(EDSS)评分。采用逻辑回归模型和 Spearman 相关分析,分析危险因素与发病时初始 EDSS 评分的相关性。采用受试者工作特征(ROC)曲线分析尿酸和同型半胱氨酸(Hcy)水平对 MOGAD 发病时神经功能障碍严重程度的预测能力。

结果

本研究纳入了 67 例(女性 34 例,男性 33 例)首发 MOGAD 患者。发病年龄的平均值为 26.43±18.22 岁(范围:3-79 岁)。<18 岁患者中最常见的首发症状为视力丧失(36.0%)和恶心呕吐(24.0%),最常见的疾病谱为急性播散性脑脊髓炎(ADEM)(40.0%)。≥18 岁患者中最常见的首发症状为视力丧失(35.7%)、感觉异常(33.3%)和瘫痪(26.2%),最常见的疾病谱为视神经炎(35.7%)。皮质灰质/皮质下白质病变是儿科和成年患者最常见的病变。尿酸[比值比(OR)=1.014;95%置信区间(CI)=1.006-1.022;P=0.000]和血清 Hcy(OR=1.125;95%CI=1.017-1.246;P=0.023)水平与发病时神经功能障碍的严重程度显著相关。尿酸水平(r=0.2583;P=0.035)和 Hcy 水平(r=0.3971;P=0.0009)与初始 EDSS 评分呈正相关。尿酸和 Hcy 水平的 ROC 曲线下面积分别为 0.7775(95%CI=0.6617-0.8933;P<0.001)和 0.6767(95%CI=0.5433-0.8102,P=0.014)。

结论

不同年龄患者的 MOGAD 临床表型不同。<18 岁患者最常见的疾病谱为 ADEM,而≥18 岁患者中视神经炎较为常见。尿酸和 Hcy 水平是首发 MOGAD 患者发病时神经功能障碍严重程度的危险因素。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9051/8714638/89ac22c813d6/fimmu-12-752557-g001.jpg

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