小儿发病多发性硬化症:人口统计学、症状和疾病进展。

IF 7.5 1区 医学 Q1 CLINICAL NEUROLOGY
David Ellenberger, Peter Flachenecker, Judith Haas, Kerstin Hellwig, Dieter Pöhlau, Alexander Stahmann, Clemens Warnke, Uwe K Zettl, Paulus Stefan Rommer
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引用次数: 0

摘要

背景:多发性硬化症(MS)是青壮年最常见的神经免疫疾病。18岁前临床发病(儿科发病多发性硬化症(POMS))的数据有限。方法:这项观察性研究提供了bbb1000poms与成人发病MS (AOMS)的数据,并使用广义加性模型和相关混杂因素调整分析了患者的诊断延迟、初始症状和长期结局。结果:结果显示诊断延迟,并且与AOMS相比,POMS的女性比例更高。感觉(57%)和视觉(48%)障碍是POMS最常见的初始症状。在临床发病后的前15年内,POMS的复发率高于AOMS。15年达到扩展残疾状态量表(EDSS) 3.0分的患者比例在POMS中(41%)低于AOMS(年龄依赖性,48%-71%)。在40岁以后的POMS患者中观察到EDSS的平台期,而在AOMS患者中未见。这个平台期是导致POMS和AOMS之间EDSS评分随年龄增长而趋于平衡的原因。临床发病时的小脑和多症状症状以及男性是POMS患者较高EDSS评分的预测因子,而在AOMS患者中,锥体功能障碍是较差结果的预测因子。结论:这项迄今为止规模最大、时间最长的POMS随访研究显示,女性更有可能在年轻时患上MS,并经历与男性不同的症状。POMS患者复发率较高,但复发率恢复较快,在以后的生活中病情也较为稳定。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Paediatric-onset multiple sclerosis: demographics, symptoms and disease progression.

Background: Multiple sclerosis (MS) is the most common neuroimmunological disease in young adults. Data on its clinical onset before the age of 18 (paediatric-onset MS (POMS)) are limited.

Methods: This observational study present data on >1000 POMS compared with adult-onset MS (AOMS) and analysed patients regarding diagnostic delay, initial symptoms and long-term outcome using generalised additive models and adjustment for relevant confounders.

Results: The results showed a diagnostic delay and a higher proportion of women with POMS vs AOMS. Sensory (57%) and visual (48%) disturbances were the most common initial symptoms of POMS. Relapse rates were higher in POMS than in AOMS within the first 15 years after the clinical onset. The proportion of patients reaching an Expanded Disability Status Scale (EDSS) score of 3.0 by 15 years was lower in POMS (41%) than in AOMS (age-dependent, 48%-71%). A plateau phase in EDSS was observed in patients with POMS after age 40, which was not seen in those with AOMS. This plateau phase was responsible for the equalisation of the EDSS score with advanced age between POMS and AOMS. Cerebellar and polysymptomatic symptoms at clinical onset and male sex were predictors of higher EDSS scores in POMS, whereas in AOMS, pyramidal dysfunction was a predictor of worse outcomes.

Conclusions: This largest and longest follow-up study of POMS to date revealed that women are more likely to develop MS at younger ages and experience different symptoms than men. Patients with POMS tend to have higher relapse rates but may recover more quickly from relapses and experience a more stable disease course later in life.

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来源期刊
CiteScore
15.70
自引率
1.80%
发文量
888
审稿时长
6 months
期刊介绍: The Journal of Neurology, Neurosurgery & Psychiatry (JNNP) aspires to publish groundbreaking and cutting-edge research worldwide. Covering the entire spectrum of neurological sciences, the journal focuses on common disorders like stroke, multiple sclerosis, Parkinson’s disease, epilepsy, peripheral neuropathy, subarachnoid haemorrhage, and neuropsychiatry, while also addressing complex challenges such as ALS. With early online publication, regular podcasts, and an extensive archive collection boasting the longest half-life in clinical neuroscience journals, JNNP aims to be a trailblazer in the field.
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