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发病时,年幼的多发性硬化症患儿的脑脊液具有明显的炎症特征。

Younger children with MS have a distinct CSF inflammatory profile at disease onset.

机构信息

UCSF Regional Pediatric MS Center, 350 Parnassus Ave., Suite 908, San Francisco, CA 94117, USA.

出版信息

Neurology. 2010 Feb 2;74(5):399-405. doi: 10.1212/WNL.0b013e3181ce5db0.

Abstract

BACKGROUND

The clinical and MRI presentation differs between earlier- and later-onset pediatric multiple sclerosis (MS), whereas the effect of age on the CSF inflammatory profile is unknown and may contribute to delayed diagnosis.

OBJECTIVES

To compare the CSF cellular and immunoglobulin G (IgG) profiles between earlier- and later-onset pediatric MS.

METHODS

We queried the databases of 6 pediatric MS centers for earlier-onset (onset <11 years) and later-onset (> or = 11 and <18 years) patients with MS or clinically isolated syndrome who underwent CSF analysis within the first 3 months of presentation (observational study). We compared CSF white blood cell (WBC) differential count, IgG index, and IgG oligoclonal bands between age groups.

RESULTS

We identified 40 earlier-onset (mean age at onset = 7.2 +/- 2.7 years, 60% females) and 67 later-onset pediatric MS patients (15.1 +/- 1.7 years, 63% females). Although WBC count tended to be higher in earlier-onset patients (median = 9/mm(3) [0-343] vs 6 [0-140], p = 0.15), they had a lower proportion of lymphocytes (70% [0-100] vs 93% [0-100] of WBCs, p = 0.0085; difference = +3% per 1-year increase of age, p = 0.0011) and higher proportion of neutrophils than later-onset patients (0.5% [0-75] vs 0% [0-50] of WBCs, p = 0.16; difference = -1% per 1-year increase of age, p = 0.033). In earlier-onset disease, fewer patients had an elevated IgG index than in the later-onset group (35% vs 68% of patients, p = 0.031).

CONCLUSION

Age modifies the CSF profile at pediatric multiple sclerosis (MS) onset, which may mislead the diagnosis. Our findings suggest an activation of the innate rather than the adaptive immune system in the earlier stages of MS or an immature immune response.

摘要

背景

早发型和晚发型儿科多发性硬化症(MS)的临床和 MRI 表现不同,而年龄对 CSF 炎症特征的影响尚不清楚,可能导致诊断延迟。

目的

比较早发型和晚发型儿科 MS 患者 CSF 的细胞和免疫球蛋白 G(IgG)谱。

方法

我们在 6 个儿科 MS 中心的数据库中查询了在发病后 3 个月内进行 CSF 分析的 MS 或临床孤立综合征的早发型(发病<11 岁)和晚发型(> = 11 岁且<18 岁)患者的资料(观察性研究)。我们比较了年龄组之间 CSF 白细胞(WBC)差异计数、IgG 指数和 IgG 寡克隆带。

结果

我们确定了 40 例早发型(发病时平均年龄=7.2 ± 2.7 岁,60%女性)和 67 例晚发型儿科 MS 患者(15.1 ± 1.7 岁,63%女性)。尽管早发型患者的 WBC 计数较高(中位数=9/mm³[0-343]比 6/mm³[0-140],p=0.15),但他们的淋巴细胞比例较低(70%[0-100]比 93%[0-100]的 WBC,p=0.0085;差异为每增加 1 岁年龄增加 3%,p=0.0011),中性粒细胞比例较高(0.5%[0-75]比 0%[0-50]的 WBC,p=0.16;差异为每增加 1 岁年龄降低 1%,p=0.033)。在早发型疾病中,与晚发型组相比,IgG 指数升高的患者较少(35%比 68%的患者,p=0.031)。

结论

年龄改变了儿科 MS 发病时的 CSF 特征,可能导致诊断错误。我们的研究结果表明,在 MS 的早期阶段,固有免疫而不是适应性免疫系统被激活,或者是不成熟的免疫反应。

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