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儿童多发性硬化症青春期前的临床表现。

Clinical presentation of pediatric multiple sclerosis before puberty.

机构信息

Department of Pediatrics and Pediatric Neurology, University Medical Center, Georg August University Göttingen, Göttingen, Germany.

出版信息

Eur J Neurol. 2014 Mar;21(3):441-6. doi: 10.1111/ene.12327. Epub 2013 Dec 16.

Abstract

BACKGROUND AND PURPOSE

Multiple sclerosis (MS) onset before puberty is extremely rare and establishment of diagnosis is often difficult due to atypical presentation. The study aims to identify the typical presentation of MS in this age group.

METHODS

Pediatric MS patients were identified from the database of the Center for Multiple Sclerosis in Childhood and Adolescence at the University Medical Center Göttingen, Germany. Inclusion criteria were a relapsing-remitting initial disease course and minimum disease duration of 4 years.

RESULTS

Forty-seven pre-pubertal (<11 years) and 41 post-pubertal (14-16 years) MS patients were compared. Before puberty an even gender ratio was found. The pre-pubertal patients were more likely to have a polysymptomatic severe first attack with motor and brainstem involvement, sphincter dysfunction, cognitive disturbances and milder residual neurological sequelae after the first episode whilst the post-pubertal patients predominantly presented with optic neuritis and sensory symptoms. The initial symptom pattern prevailed over the first 2 years of disease. Presentation of pre-pubertal boys and girls did not differ significantly.

CONCLUSIONS

To facilitate early diagnosis it is important to recognize that pre-pubertal MS presents with a specific pattern of symptoms that is maintained over the first two disease years.

摘要

背景与目的

青春期前发病的多发性硬化症(MS)极其罕见,由于表现不典型,诊断往往较为困难。本研究旨在确定该年龄段 MS 的典型表现。

方法

从德国哥廷根大学医学中心儿童多发性硬化症中心的数据库中确定儿科 MS 患者。纳入标准为复发缓解型初始病程和至少 4 年的疾病持续时间。

结果

比较了 47 名青春期前(<11 岁)和 41 名青春期后(14-16 岁)MS 患者。青春期前患者的性别比例较为均衡。青春期前患者更可能出现多症状严重首发,伴有运动和脑干受累、括约肌功能障碍、认知障碍和首发后较轻的神经后遗症,而青春期后患者主要表现为视神经炎和感觉症状。初始症状模式在疾病的前 2 年占主导地位。青春期前男孩和女孩的表现没有显著差异。

结论

为了促进早期诊断,重要的是要认识到青春期前 MS 具有特定的症状模式,这种模式在最初的两年疾病中保持不变。

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