Phenotypic characteristics of myelin oligodendrocyte glycoprotein antibody-associated disease in children: a single-center, retrospective study.

Phenotypic characteristics of myelin oligodendrocyte glycoprotein antibody-associated disease in children: a single-center, retrospective study.
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DOI:
10.3389/fneur.2023.1188323
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发表时间:
2023
影响因子:
3.4
通讯作者:
--
中科院分区:
医学3区
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--
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目的分析髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)不同临床表型患儿的临床特点及随访资料。回顾性分析我国74例不同表型MOGAD患儿的基本人口学、临床特征、实验室及影像学检查结果及随访资料。该队列的男女比例为1:1.39。MOGAD的临床表型包括急性播散性脑脊髓炎(ADEM, n = 37)、脑炎(n = 11)、视神经炎(ON, n = 9)、视神经脊髓炎视谱障碍(NMOSD, n = 9)、横贯性脊髓炎(TM, n = 6)、脑白质营养不良样表现(n = 1)、脑膜炎(n = 1)。平均发病年龄为86个月。ADEM患者脑脊液中白细胞数量显著高于ON患者,低于TM患者(p < 0.05)。所有患者病原菌检出率为36.5%。复发17例(23%),以NMOSD和TM患者复发率最高。复发患者的中位年龄明显高于无复发患者(109.00 vs 82.44个月,p < 0.05)。复发患者的男女比例为1:4.67,与首发患者比较差异有统计学意义(p < 0.05)。该队列中MOGAD最常见的临床表型是ADEM和脑炎。MOGAD的复发率可能与年龄和性别有关,女性复发率较高。这些发现为进一步探索不同MOGAD表型的特征提供了基础。
To analyze the clinical characteristics and follow-up data of children with different clinical phenotypes of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). The basic demographic and clinical features, laboratory and imaging examination results, and follow-up data of 74 Chinese children with different phenotypes of MOGAD were retrospectively reviewed and analyzed. The male-to-female ratio in this cohort was 1:1.39. The clinical phenotypes of MOGAD included acute disseminated encephalomyelitis (ADEM; n = 37), encephalitis (n = 11), optic neuritis (ON, n = 9), neuromyelitis optica spectrum disorder (NMOSD; n = 9), transverse myelitis (TM; n = 6), leukodystrophy-like manifestations (n = 1), and meningitis (n = 1). The mean age of disease onset was 86 months. The number of leukocytes in the cerebrospinal fluid of patients with ADEM was significantly higher than that in patients with ON but lower than that in patients with TM (p < 0.05). The pathogen detection rate among all patients was 36.5%. Recurrence occurred in 17 patients (23%), with the highest recurrence rate in patients with NMOSD and TM. Patients with recurrence had a significantly higher median age than those without any recurrence (109.00 vs. 82.44 months, p < 0.05). The male-to-female ratio in patients with recurrence was 1:4.67, which differed significantly from that at first onset (p < 0.05). The most common clinical phenotypes of MOGAD in this cohort were ADEM and encephalitis. Recurrence of MOGAD may be related to age and sex, with a higher recurrence rate observed in females. These findings provide a basis for further exploration of the characteristics of different MOGAD phenotypes.
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发表时间: 2007-02
期刊: The American journal of pathology
影响因子: --
作者:
Burrer R;Buchmeier MJ;Wolfe T;Ting JP;Feuer R;Iglesias A;von Herrath MG
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期刊: NEUROPEDIATRICS
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影响因子: 3.1
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