Department of Neurosurgery, Aalborg University Hospital, Aalborg, Denmark.
Department of Orthopaedic Surgery, Odense University Hospital, Odense, Denmark.
Eur J Neurol. 2020 Dec;27(12):2446-2452. doi: 10.1111/ene.14439. Epub 2020 Aug 8.
The incidence of moyamoya disease (MMD) in Europe is not well known. In those affected, the risk of brain hemorrhage is considered low. The present study aimed to investigate the incidence and clinical presentation of MMD in the Danish population.
Eligible patients were identified in the Danish National Patient Register from 1994 to 2017. We collected clinical and radiological data from individual patient records from neurological, neurosurgical and paediatric units across Denmark. The diagnosis was validated according to established criteria. We also extracted basic demographic data on the cohort from the Danish Civil Registration System.
A total of 52 patients fulfilled the diagnostic criteria for MMD. Most patients were native Danes and only 15% had an East Asian background. The ratio of female to male patients was 1.8, and the incidence had two peaks: one in childhood and another in young middle age. Until 2007, MMD was only diagnosed sporadically. From 2008 onwards, the incidence rate was 0.07 per 100 000 person-years (95% confidence interval 0.05-0.09 per 100 000 person-years). The most common mode of presentation was ischaemic stroke (33%), followed by hemorrhage (23%), headache (17%) and transient ischaemic attack (14%).
Moyamoya disease is rare in Denmark, but is associated with a considerable risk of hemorrhage. Thus, MMD should be considered in the evaluation for ischaemic as well as hemorrhagic stroke paediatric and middle-aged Caucasians.
欧洲的 moyamoya 病（MMD）发病率尚不清楚。在受影响的患者中，脑出血的风险被认为较低。本研究旨在调查丹麦人群中 MMD 的发病率和临床表现。
从 1994 年至 2017 年，在丹麦国家患者登记处确定符合条件的患者。我们从丹麦各地的神经科、神经外科和儿科单位的患者病历中收集了临床和影像学数据。根据既定标准对诊断进行了验证。我们还从丹麦民事登记系统中提取了该队列的基本人口统计学数据。
共有 52 名患者符合 MMD 的诊断标准。大多数患者是丹麦本地人，只有 15%有东亚背景。女性与男性患者的比例为 1.8，发病率有两个高峰：一个在儿童期，另一个在年轻的中年期。直到 2007 年，MMD 才偶有诊断。从 2008 年开始，发病率为 0.07/100 000 人年（95%置信区间为 0.05-0.09/100 000 人年）。最常见的表现形式是缺血性中风（33%），其次是出血（23%）、头痛（17%）和短暂性脑缺血发作（14%）。
丹麦的 moyamoya 病罕见，但与相当大的出血风险相关。因此，在评估缺血性和出血性中风的儿科和中年白种人时，应考虑 MMD。
