庫賈氏病為罕見的進行性神經退化性疾病,全世界發生率為每年每百萬人口0.5-1人,從出現臨床症狀至死亡約為4.5個月-14個月,目前無有效治療方法。我國自1996年開始持續監測,自2008年後通報病例數激增,年發率及相關流行病學有顯著改變,本研究目的為分析我國2001年~2010年台灣地區庫賈氏病流行分布狀況及相關危險因子,作為防治策略制定的依據,以使監測及防治機制更臻完善。應用回溯性病例對照研究法,以2001年~2010年通報疾病管制局經審查研判為可能、極可能及確定157例病例中118例為實驗組,審查為排除病例中108例為對照組,研究疾病發生之危險因子。結果發現我國年發生率為每百萬人口0.3~0.8人,有明顯上升趨勢,存活分析平均為537天,發病年齡每上升1歲,存活風險增加1.064倍,影響罹病之危險因子以至國外旅遊者、喝酒及家族罹患失智症顯著增加。所以衛生主管機關宜及早規劃因病例增加及存活時間增加所衍生之長期照護需求及落實相關感染控制措施。
Abstract Creutzfeldt-Jakob disease is a rare neurodegenerative disease, the world incidence rate is 0.5 people per million population, about 4.5-14 months from the onset of clinical symptoms to death, currently no effective treatment. Taiwan began monitoring since 1996. Surge in the number of cases notified in 2008, the annual incidence rates and the epidemiology of Creutzfeldt-Jakob disease change significantly. The purpose of this study for analysis of 2001 to 2010 of Creutzfeldt-Jakob disease epidemic distribution and risk factors, as the basis for the control strategies developed to enable monitoring and control mechanisms to be perfected. Application of retrospective case control study, subjects were informed of the case of the Centers for Disease Control from 2001 to 2010, which judged to possible, probable and definite the case of 118 cases for the experimental group, judged to exclude the cases of 108 cases in the control group. The results showed that the annual rate were from 0.3 to 0.8 per million population, there is a clear upward trend. Survival analysis is an average of 537 days, age of onset for each increase of 1-year-old, survival increased risk of 1.064 times. The risk factors of morbidity as well as foreign tourists, drinking, and family dementia increased significantly. Therefore, health authorities should be early planning derived from long-term care, and strengthening infection control measures.