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土耳其某转诊中心儿科和成人神经科对小儿多发性硬化不良结局的随访。

Unfavorable outcome of pediatric onset multiple sclerosis: Follow-up in the pediatric and adult neurology departments of one referral center, in Turkey.

机构信息

Department of Neurology, Baskent University Faculty of Medicine, Mareşal Fevzi Çakmak Cad. 10.sok No: 45, Bahçelievler, Ankara, Turkey.

Department of Neurology, Hacettepe University Faculty of Medicine, Hacettepe Üniversitesi Tıp Fakültesi, 06100 Sıhhiye Ankara, Turkey.

出版信息

Mult Scler Relat Disord. 2016 Sep;9:1-4. doi: 10.1016/j.msard.2016.06.002. Epub 2016 Jun 8.

Abstract

BACKGROUND

The prevalence of MS starting under 18 years of age ranges between 2-10% of the total MS population.

OBJECTIVE

We aimed to examine the clinical and long term follow-up data of pediatric-onset cases in our institutional MS database.

METHOD

We evaluated the clinical data from the MS database of the Departments of Neurology and Pediatric Neurology of Hacettepe University Hospital.

RESULTS

The clinical features of 74 patients who had experienced the first attack before age 18 years comprised 3.9% of our MS population. Median age at onset was 15 (3, 5-17, IQR=3.63) years, and female: male ratio was 2.4. The most frequent symptom at onset was brainstem/cerebellar dysfunction (32.4%). Seventy two patients (97.3%) initially had relapsing remitting course and in the follow-up, 17 (23%) of them developed secondary progressive (SP) course. The median interval to develop SPMS course was 10 (5-21, IQR=8) years. At the last visit, median disease duration was 6.67 (0.83-25, IQR=9.06) years, 41 (55.4%) of them had EDSS of ≥4.

CONCLUSION

These findings illustrate the profile of our pediatric MS patients: almost all are relapsing-remitting initially; about one fourth become secondarily progressive in 10 years, and about half acquire disability EDSS ≥4 in mean 8 years.

摘要

背景

18 岁以下发病的多发性硬化症(MS)患病率占总 MS 人群的 2-10%。

目的

我们旨在检查我们机构 MS 数据库中儿科发病病例的临床和长期随访数据。

方法

我们评估了来自哈塞特佩大学医院神经内科和儿科神经内科 MS 数据库的临床数据。

结果

74 例年龄在 18 岁以下首次发病的患者的临床特征占我们 MS 人群的 3.9%。发病中位年龄为 15 岁(3、5-17、IQR=3.63),男女比例为 2.4。首发最常见的症状是脑干/小脑功能障碍(32.4%)。72 例(97.3%)患者最初为复发缓解型病程,在随访中,17 例(23%)患者发展为继发进展型(SP)病程。发展为继发进展型多发性硬化症的中位时间为 10 年(5-21,IQR=8)。最后一次就诊时,中位病程为 6.67 年(0.83-25,IQR=9.06),41 例(55.4%)患者的 EDSS 评分≥4。

结论

这些发现说明了我们儿科 MS 患者的情况:几乎所有患者最初均为复发缓解型;约四分之一在 10 年内进展为继发进展型,约一半在平均 8 年内获得残疾 EDSS≥4。

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