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斯洛文尼亚儿科人群多发性硬化症的流行病学和临床特征:一项描述性全国性研究。

Epidemiological and clinical characteristics of multiple sclerosis in paediatric population in Slovenia: A descriptive nation-wide study.

机构信息

Departments of Child, Adolescent and Developmental Neurology, University Children's Hospital, University Medical Centre Ljubljana, Slovenia.

Department of Neurology, University Medical Centre Ljubljana, Slovenia.

出版信息

Mult Scler Relat Disord. 2017 Nov;18:56-59. doi: 10.1016/j.msard.2017.09.017. Epub 2017 Sep 21.

Abstract

BACKGROUND

Although multiple sclerosis usually affects young adults, paediatric-onset multiple sclerosis (pMS) is increasingly recognized in the past ten years. The aim of the present study was to evaluate the incidence of pMS in Slovenia and to characterize the clinical, laboratory and neuroradiological characteristics of pMS at the disease onset.

METHODS

We performed a national retrospective descriptive study including all patients diagnosed with pMS between January 1992 and June 2017. We reviewed data of all patients younger than 18 years at the first demyelinating event.

RESULTS

The estimated incidence of pMS was 0.66/100,000 children per year. We included 61 patients (77% were female) with a median age at diagnosis of 16.3 years. In 4 patients, onset of pMS was before the age of 12 years old (childhood-onset pMS). Relapsing-remitting multiple sclerosis was most prevalent, with only 2 patients presenting a primary progressive pMS. Polysymptomatic pMS was found at onset in 59% of patients and monosymptomatic in 41%. In the cerebrospinal fluid study, 88% of patients had positive oligoclonal bands. Brain magnetic resonance imaging studies showed a predominant supratentorial involvement (100% of patients).

CONCLUSION

The clinical pattern of pMS in our cohort of patients was characterized by polysymptomatic presentation and predominantly sensory symptoms at onset, developing a relapsing-remitting pMS pattern. It is important to gather more information about the incidence of pMS and its initial presentation and clinical course to improve early recognition and appropriate initiation of immunomodulatory treatment.

摘要

背景

多发性硬化症通常影响年轻人,但过去十年中,儿科发病型多发性硬化症(pMS)的发病率越来越高。本研究的目的是评估斯洛文尼亚 pMS 的发病率,并描述 pMS 发病时的临床、实验室和神经影像学特征。

方法

我们进行了一项全国性回顾性描述性研究,纳入了 1992 年 1 月至 2017 年 6 月期间诊断为 pMS 的所有患者。我们回顾了首次脱髓鞘事件时年龄小于 18 岁的所有患者的数据。

结果

pMS 的估计发病率为每年每 10 万儿童 0.66 例。我们纳入了 61 名患者(77%为女性),诊断时的中位年龄为 16.3 岁。4 名患者 pMS 发病年龄在 12 岁之前(儿童发病型 pMS)。最常见的是复发缓解型多发性硬化症,只有 2 名患者表现为原发性进展型 pMS。59%的患者在发病时表现为多症状性,41%为单症状性。在脑脊液研究中,88%的患者寡克隆带阳性。脑磁共振成像研究显示,100%的患者存在明显的幕上受累。

结论

我们的患者队列中 pMS 的临床特征为多症状表现,发病时主要为感觉症状,发展为复发缓解型 pMS。重要的是,需要收集更多关于 pMS 的发病率及其初始表现和临床病程的信息,以提高早期识别和适当启动免疫调节治疗。

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