Pohl Daniela, Hennemuth Isabell, von Kries Rüdiger, Hanefeld Folker
Department of Paediatrics and Paediatric Neurology, Georg-August-University, Robert-Koch-Strasse 40, 37075, Göttingen, Germany.
Eur J Pediatr. 2007 May;166(5):405-12. doi: 10.1007/s00431-006-0249-2. Epub 2007 Jan 12.
The aim of this study was to evaluate the incidence of paediatric multiple sclerosis (MS) and acute disseminated encephalomyelitis (ADEM) in Germany. In a prospective nationwide survey carried out between 1997 and 1999, all registered new cases of paediatric MS and ADEM with an onset before the age of 16 years were evaluated using a standardised questionnaire. A total of 132 patients with suspected or definite MS and 28 patients with an assumed diagnosis of ADEM were reported. Among these, 82% of the MS patients were 10 years of age or older, as opposed to 18% in the ADEM-cohort. The female-to-male ratio was 1.2:1 in the MS-cohort and 0.8:1 in the ADEM-cohort. Manifestation was polysymptomatic in 67% of the MS patients compared to 86% of the ADEM patients. The most frequent primary symptoms in the MS-cohort were cerebellar (44%), sensory (39%) or visual (36%), followed by brainstem (30%), pyramidal (29%) and cerebromental (22%) complaints.
The incidence of paediatric MS in Germany is more than fourfold higher than that of paediatric ADEM; in addition, it shows a strikingly different age-distribution. With an estimated minimum of 50 new cases per year, the incidence of paediatric MS in Germany is much more frequent than previously believed.
本研究的目的是评估德国儿童多发性硬化症(MS)和急性播散性脑脊髓炎(ADEM)的发病率。在1997年至1999年进行的一项全国性前瞻性调查中,使用标准化问卷对所有登记的16岁之前发病的儿童MS和ADEM新病例进行了评估。共报告了132例疑似或确诊为MS的患者以及28例假定诊断为ADEM的患者。其中,82%的MS患者年龄在10岁及以上,而ADEM队列中这一比例为18%。MS队列中女性与男性的比例为1.2:1,ADEM队列中为0.8:1。67%的MS患者表现为多症状,而ADEM患者中这一比例为86%。MS队列中最常见的主要症状是小脑症状(44%)、感觉症状(39%)或视觉症状(36%),其次是脑干症状(30%)、锥体束症状(29%)和大脑症状(22%)。
德国儿童MS的发病率比儿童ADEM高出四倍多;此外,其年龄分布也显著不同。德国儿童MS的发病率估计每年至少有50例新病例,比之前认为的要频繁得多。