Lindberg Christopher, Bjerkne Fredrik
Neuromuscular Centre, Department of Neurology, Sahlgrenska University Hospital, 413 45 Gothenburg, Sweden.
Neuromuscular Centre, Department of Neurology, Sahlgrenska University Hospital, 413 45 Gothenburg, Sweden.
Neuromuscul Disord. 2017 Feb;27(2):159-162. doi: 10.1016/j.nmd.2016.12.005. Epub 2016 Dec 13.
Myotonic dystrophy type 1 (DM1) is the most common inherited muscle disorder in adults. The prevalence differs widely between countries, but a figure of 13/100.000 is most frequently cited. It is a multi-organ disorder classified into four categories: congenital, childhood, adult/classical and late-onset/mild. The purpose of this study was to estimate the total and age adjusted prevalence of DM1 in adults in western Sweden (the Västra Götaland Region, VGR) as well as in the city of Gothenburg and also in the VGR except Gothenburg. Patients with the diagnosis of DM1 in the VGR were traced by outpatient registers at the Neuromuscular Center, contacted by regular mail and thereafter telephone interviewed about organ manifestations in order to ascertain the age at onset and thus the disease category. Medical records were examined to obtain detail accuracy. We detected 230 adult DM1 patients in the VGR which gives a prevalence of 17.8/100.000. The prevalence of DM1 in Gothenburg was 14.1/100.000, which was significantly lower than in the remaining region which was 19.7/100.000. There was no gender difference. The age adjusted prevalence rates showed that DM1 is most prevalent in the age group 35-44 years (23.9/100.000) and 45-54 years (25.8/100.000). DM1 prevalence in the western Sweden thus seems to be somewhat higher than elsewhere in Europe, and is especially high in the less densely populated areas of the region. The disease burden in the community is larger than what was known previously.
1型强直性肌营养不良（DM1）是成人中最常见的遗传性肌肉疾病。各国的患病率差异很大，但最常引用的数字是13/100000。它是一种多器官疾病，分为四类：先天性、儿童期、成人/经典型和晚发型/轻型。本研究的目的是估计瑞典西部（韦斯特罗斯-哥德堡地区，VGR）以及哥德堡市和除哥德堡以外的VGR地区成人DM1的总患病率和年龄调整患病率。通过神经肌肉中心的门诊登记册追踪VGR地区诊断为DM1的患者，通过定期邮件联系他们，然后通过电话询问器官表现，以确定发病年龄，从而确定疾病类型。检查医疗记录以获得详细的准确性。我们在VGR地区检测到230例成人DM1患者，患病率为17.8/100000。哥德堡的DM1患病率为14.1/100000，显著低于其余地区的19.7/100000。没有性别差异。年龄调整患病率显示，DM1在35-44岁年龄组（23.9/100000）和45-54岁年龄组（25.8/100000）中最为普遍。因此，瑞典西部的DM1患病率似乎略高于欧洲其他地区，在该地区人口密度较低的地区尤其高。社区中的疾病负担比以前所知的要大。
