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克里特良性多发性硬化症。

Benign multiple sclerosis in Crete.

机构信息

Department of Neurology and the Neurology Service of the University Hospital, School of Health Sciences, University of Crete, Heraklion, Crete, Greece.

出版信息

Mult Scler. 2010 Jun;16(6):701-6. doi: 10.1177/1352458510364631. Epub 2010 Mar 17.

Abstract

Our objective was to study multiple sclerosis on Crete, an island of 0.6 million inhabitants sharing a similar genetic background and the same environment. Case ascertainment was achieved using the MS Epidemiology Program Project of Crete. The diagnosis and classification of multiple sclerosis were made by established clinical and magnetic resonance imaging criteria, and disease evolution was assessed by periodic evaluations. Thorough clinical and laboratory evaluations were conducted; a detailed history, including a questionnaire of 36 items, was taken. Data obtained were analysed for possible interaction with disease prognosis. We identified 587 cases of multiple sclerosis (F:M = 1.6), >90% of which were of Cretan origin from both parental lines. Age at onset was 31.5 +/- 10.3 years (mean +/- SD) and disease duration 12.7 +/- 9.1 years. About 84.6% had relapsing remitting multiple sclerosis, 9.4% primary progressive multiple sclerosis and 6% clinically isolated syndrome. Nearly 40% of our multiple sclerosis patients with disease duration >10 years (mean = 16.2 +/- 5.3 years) remained with no or mild disability (Expanded Disability Status Scale [EDSS] < or =3). Also, about 30% of patients with relapsing remitting multiple sclerosis showed benign disease evolution (EDSS < or =3) more than 20 years (mean = 24.0 +/- 3.3) after onset. Factors predisposing to benign multiple sclerosis included younger age at onset, shorter disease duration and a lower number of relapses. We conclude that a substantial proportion of patients with multiple sclerosis from Crete follow a rather benign disease course, and this may relate to the genetic background of the population and/or to environmental factors.

摘要

我们的目的是研究克里特岛上的多发性硬化症,克里特岛是一个拥有 60 万居民的岛屿,具有相似的遗传背景和相同的环境。通过克里特多发性硬化症流行病学项目计划实现病例确定。多发性硬化症的诊断和分类采用既定的临床和磁共振成像标准,通过定期评估评估疾病的演变。进行了彻底的临床和实验室评估;进行了详细的病史评估,包括 36 个项目的问卷。分析了获得的数据,以研究其与疾病预后的可能相互作用。我们确定了 587 例多发性硬化症病例(女性与男性比例为 1.6),其中 90%以上为来自双亲双方的克里特岛本地人。发病年龄为 31.5 +/- 10.3 岁(平均值 +/- 标准差),病程为 12.7 +/- 9.1 年。约 84.6%的患者为复发缓解型多发性硬化症,9.4%为原发性进行性多发性硬化症,6%为临床孤立综合征。在我们的多发性硬化症患者中,病程>10 年(平均 = 16.2 +/- 5.3 年)的患者中,约有 40%没有或仅有轻度残疾(扩展残疾状况量表 [EDSS] <或=3)。此外,约 30%的复发缓解型多发性硬化症患者在发病后 20 多年(平均 = 24.0 +/- 3.3)表现出良性疾病演变(EDSS <或=3)。导致多发性硬化症良性演变的因素包括发病年龄较小、病程较短和发作次数较少。我们得出结论,克里特岛的多发性硬化症患者中有相当一部分患者的疾病病程相当良性,这可能与该人群的遗传背景和/或环境因素有关。

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