Department of Primary Care and Population Health, University of Nicosia Medical School, Nicosia, Cyprus.
Neurology Clinic D, The Cyprus Institute of Neurology and Genetics, Nicosia, Cyprus.
PLoS One. 2019 Sep 6;14(9):e0220246. doi: 10.1371/journal.pone.0220246. eCollection 2019.
Amyotrophic lateral sclerosis (ALS) is a rare, progressive neurodegenerative disease. There is heterogeneity of clinical phenotypes while a clinical characterization of ALS in Cyprus is still lacking. The aim of this 30-year retrospective study of ALS in Cyprus is to determine the demographic characteristics of patients, the clinical features of the disease, the uptake of supportive therapies and factors influencing survival.
All ALS patients seen at the Cyprus Institute of Neurology and Genetics from January 1985 until July 2015 were included. Medical records of eligible patients were used for data extraction and compilation of an ALS database. Clinical features were compared between gender categories using univariate tests, while survival was assessed using Kaplan-Meier curves. Cox proportional hazards models were used to identify prognostic factors for survival.
One hundred and seventy-nine ALS patients were included in the study, of whom 7 had a positive family history. Most clinical characteristics of ALS did not differ from what is observed in other European countries. However, some clinical characteristics were unique to our population, such as an increased acceptability and utilisation of supportive treatments such as gastrostomy.
Overall, clinical characteristics of patients with ALS in the Republic of Cyprus do not differ from other European counties. Our study demonstrates a high acceptance and utilisation of supportive interventions enhancing survival, in the context of a multidisciplinary approach offered in the single tertiary centre that services the whole Cypriot ALS population. The findings of this paper are of value to the health professionals treating ALS in Cyprus.
肌萎缩侧索硬化症（ALS）是一种罕见的进行性神经退行性疾病。尽管塞浦路斯的 ALS 临床特征尚不清楚，但临床表型存在异质性。本研究对塞浦路斯 30 年来的 ALS 病例进行回顾性分析，旨在确定患者的人口统计学特征、疾病的临床特征、支持性治疗的采用情况以及影响生存的因素。
本研究纳入了 1985 年 1 月至 2015 年 7 月期间在塞浦路斯神经病学和遗传学研究所就诊的所有 ALS 患者。使用合格患者的病历记录来提取数据并编制 ALS 数据库。使用单变量检验比较性别组之间的临床特征，使用 Kaplan-Meier 曲线评估生存情况。使用 Cox 比例风险模型确定影响生存的预后因素。
本研究共纳入了 179 例 ALS 患者，其中 7 例有阳性家族史。ALS 的大多数临床特征与其他欧洲国家观察到的情况并无不同。然而，我们的人群中存在一些独特的临床特征，例如对胃造口术等支持性治疗的接受度和使用率较高。
总体而言，塞浦路斯共和国 ALS 患者的临床特征与其他欧洲国家无差异。本研究表明，在为整个塞浦路斯 ALS 患者服务的单一三级中心提供的多学科方法背景下，支持性干预措施的接受度和使用率较高，从而提高了生存率。本文的研究结果对在塞浦路斯治疗 ALS 的医疗专业人员具有重要价值。
