台灣肌萎縮性脊髓側索硬化症的發生率、盛行率及死亡率

Incidence, prevalence and mortality of amyotrophic lateral sclerosis in Taiwan

目標：肌萎縮性脊髓側索硬化症（Amyotrophic Lateral Sclerosis, ALS）為一重大疾病，監 測其基礎流行病學指標以掌握此疾病的流行趨勢至為重要。本研究分年度進行台灣的ALS發生 率、盛行率及死亡率等統計。方法：本回顧性世代研究從重大傷病資料庫納入1999年1月1日至 2017年12月31日間新診斷ALS案例，並串連台灣全民健保資料庫與全國死因資料檔進行分析。 結果：2008-2015年逐年的ALS年齡標準化發生率約在每10萬人0.33-0.44人間；年齡標準化盛 行率從2008年的1.54人上升至2015年的2.31人（每10萬人）。1999-2016年期間在初診日後的5 年內有很高的死亡率（年粗死亡率14.7%~19.7%）。另在僅納入確定死亡個案的分析中，經氣 切、非侵入型呼吸器使用或胃造口手術的個案其平均存活期則顯著提高（分別為4.92年比2.66 年，3.56年比2.45年及3.75年比3.02年）。結論：延續1999-2007年對台灣ALS流行病學之研究， 本研究顯示於2008-2015年的逐年發生率趨於穩定，而逐年盛行率有增加的趨勢。初診日後5年 內有很高的死亡率，而氣切、非侵入型呼吸器及胃造口可延長病患的存活時間。（台灣衛誌 2020；39(6)：632-642）

Objectives: Amyotrophic lateral sclerosis (ALS) is a serious disease. Therefore, monitoring basic epidemiological indicators for clarifying the epidemic trend is crucial. Yearly epidemiological statistical data were determined to assess the incidence, prevalence, and mortality of ALS in Taiwan. Methods: In this population-based retrospective cohort study, patients who were newly diagnosed with ALS from January 1, 1999, to December 31, 2017, were enrolled from the Registry for Catastrophic Illness Patient Database. Medical claims from the National Health Insurance Research Database and national mortality database were linked for further analysis. Results: The age-standardized incidence rate of ALS in 2008-2015 was approximately 0.33 to 0.44 per 100,000 people. The age-standardized prevalence rate increased from 1.54 in 2008 to 2.31 in 2015 (per 100,000 people). High mortality rate (annual crude mortality rate = 14.7%–19.7%) was observed within 5 years after the date of the first diagnosis in 1999–2016. The analysis of confirmed death cases only indicated that the average survival time improved significantly in patients who underwent tracheostomy, noninvasive ventilator use, or gastrostomy (4.92 years versus 2.66 years, 3.56 years versus 2.45 years and 3.75 years versus 3.02 years, respectively). Conclusions: Following ALS epidemiological data during 1999-2007 in Taiwan, this study revealed that the annual incidence rate of ALS in Taiwan was stable from 2008 to 2015. However, the trend of the annual prevalence rate increased gradually. The mortality rate within 5 years after the initial diagnosis was high, and treatment with tracheostomy, noninvasive ventilator use, and gastrostomy can prolong survival time. (Taiwan J Public Health. 2020;39(6):632-642)