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儿童髓鞘少突胶质细胞糖蛋白抗体相关性疾病中的脑脊液嗜酸性粒细胞。

Cerebrospinal fluid eosinophils in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease.

机构信息

Division of Neurology, Children's National Hospital, George Washington University School of Medicine and Health Sciences, Washington, DC, USA.

George Washington University School of Medicine and Health Sciences, Washington, DC, USA.

出版信息

Mult Scler Relat Disord. 2024 May;85:105526. doi: 10.1016/j.msard.2024.105526. Epub 2024 Mar 9.

DOI:10.1016/j.msard.2024.105526
PMID:38489945
Abstract

BACKGROUND

Eosinophils in cerebrospinal fluid (CSF) are an uncommon finding most often associated with parasitic infections, but have also been described in some neuroinflammatory disorders. Eosinophilic infiltration is not thought to be a typical feature of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). We aim to describe the rate of CSF eosinophil positivity in a cohort of pediatric MOGAD patients.

METHODS

Single-center retrospective chart review of pediatric MOGAD patients. Clinical and laboratory data was collected from the electronic medical record and analyzed.

RESULTS

Of 46 pediatric patients with positive serum myelin oligodendrocyte glycoprotein antibody (MOG-IgG) identified, 38 patients fulfilling internationally proposed MOGAD diagnostic criteria were included for analysis. 6 patients with MOGAD were excluded as no CSF data was available, and 2 patients with positive MOG-IgG but diagnosis more consistent with MS were excluded. Median age was 7.3 years, and 19/38 (50 %) were female. Acute disseminated encephalomyelitis (ADEM) was the most common presenting phenotype (23/38, 61 %), and other phenotypes included optic neuritis (10/38, 26 %), transverse myelitis (3/38, 8 %), and neuromyelitis optica spectrum disorder (NMOSD) (2/38, 5 %). 12 of 36 (33 %) patients with all lumbar puncture (LP) data available had CSF eosinophils present, with eosinophil mean of 3 % and range from 1 % to 18 % of CSF while blood cells.

CONCLUSION

CSF eosinophils were present in one third of pediatric MOGAD patients, which is a higher rate than previously reported in either MOGAD or aquaporin-4 antibody positive NMOSD cohorts. Understanding the CSF composition of pediatric MOGAD patients helps to facilitate more prompt diagnosis and treatment and may shed light onto underlying pathologic mechanisms of disease with the goal to inform future therapeutic targets.

摘要

背景

脑脊液(CSF)中的嗜酸性粒细胞是一种不常见的发现,最常与寄生虫感染有关,但也在一些神经炎症性疾病中有所描述。嗜酸性粒细胞浸润被认为不是髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)的典型特征。我们旨在描述一组儿科 MOGAD 患者中 CSF 嗜酸性粒细胞阳性率。

方法

对儿科 MOGAD 患者进行单中心回顾性图表审查。从电子病历中收集临床和实验室数据并进行分析。

结果

在确定的 46 例血清髓鞘少突胶质细胞糖蛋白抗体(MOG-IgG)阳性的儿科患者中,纳入了符合国际提出的 MOGAD 诊断标准的 38 例患者进行分析。有 6 例 MOGAD 患者因无 CSF 数据而被排除,有 2 例 MOG-IgG 阳性但诊断更符合 MS 的患者也被排除。中位年龄为 7.3 岁,19/38(50%)为女性。急性播散性脑脊髓炎(ADEM)是最常见的表现型(23/38,61%),其他表现型包括视神经炎(10/38,26%)、横贯性脊髓炎(3/38,8%)和视神经脊髓炎谱系障碍(NMOSD)(2/38,5%)。在有所有腰椎穿刺(LP)数据的 36 例患者中,有 12 例(33%)存在 CSF 嗜酸性粒细胞,嗜酸性粒细胞平均值为 3%,范围为 1%至 18%的 CSF 与血细胞。

结论

CSF 嗜酸性粒细胞存在于三分之一的儿科 MOGAD 患者中,这一比例高于之前报道的 MOGAD 或水通道蛋白-4 抗体阳性 NMOSD 患者。了解儿科 MOGAD 患者的 CSF 成分有助于更快速地诊断和治疗,并可能揭示疾病的潜在病理机制,从而为未来的治疗目标提供信息。

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