本研究旨在探討猝睡症患者生活品質受症狀變化及神經認知功能改變的影響情況，以及不同層次影響因素對患者生活品質的初始狀態及後續變化軌跡的影響效果。 本研究於北部一所醫學中心睡眠障礙科及兒童心智科募集確診為猝睡症之患者，經同意後進行為期五年的長期研究，募集總人數168人，完成五年資料收集人數85人。本研究使用睡眠多項檢驗（polysomnography, PSG）、多段入睡測試（multiple sleep latency test, MSLT）、人類白血球抗原檢驗（human leukocyte antigen, HLA）為基本檢驗工具，以電腦化第二版康氏持續注意力測驗（Continuous Performance Test- II）及威斯康辛卡片分類測驗（Wisconsin Card Sorting Test, WCST）為檢測神經認知功能之工具，以自填艾普渥斯嗜睡程度量表（Epworth sleepiness scale, ESS）、史丹佛睡眠問卷（Stanford sleep inventory, SSI）及簡式生活品質量表（short from-36 items of health related quality of life, SF-36）做為症狀嚴重度及生活品質的依據。資料分析以描述統計及階層線性模式（hierarchical linear models , HLM）統計方法進行。主要結果如下： 一、 猝睡症患者生活品質分為生理與心理兩個層面，患者生理層面在五年期間維持相對穩定沒有顯著變化；心理層面中之不同向度則有不同變化趨勢，心理健康與活力向度隨時間有逐漸提高的趨勢，患者此二向度生活品質接受治療後有穩定上升的趨勢，而社會功能及情緒角色限制則呈現二次方曲線變化，以及呈現先增後減的發展軌跡，患者此二向度接受治療後顯著上升，第三年後有逐年下降的趨勢。 二、 患者嗜睡程度及猝倒嚴重度變化隨時間有顯著成長軌跡，呈二次方曲線發展，轉折點在第三年，接受治療前三年症狀呈現穩定降低的軌跡，但自第三年起逐年增加，此結果與藥物治療初期症狀獲得顯著改善，後期改善幅度相對減少，及藥物效果具有關聯。 三、 個體間層次變項僅疾病持續時間、HLA對患者生活品質具顯著解釋力，其中疾病持續時間越長，患者可能發展因應症狀之策略，從而降低疾病對生活品質之衝擊。而HLA則對症狀有不同影響，HLA陽性患者初始嗜睡程度較陰性者為低，且接受治療後改善效果較陰性者顯著，猝倒嚴重度起始值較陰性者高，且接受治療後的趕善幅度較陰性者小。 四、 疾病嚴重度變化對生活品質具顯著影響，完整模式分析中，時間主效應未達顯著，但可由症狀變化及神經認知功能改變進行更佳的解釋。嗜睡程度變化僅對身體疼痛向度變化不具有解釋力外，對其餘七個向度均具顯著影響；猝倒影響層面不及嗜睡程度，但亦可解釋生理量表、生理角色限制、心理量表、心理健康、情緒角色限制、活力等向度上的變化。 五、 神經認知功能改變與否對患者生活品質具有加成效果，分析顯示患者神經認知功能改善時，其生活品質提升速率較未改善者高，影響較顯著的包括注意力、警覺度及概念反應，此結果與下視丘泌素參與的維持注意力及前額葉功能有關。 本研究依據分析結果提出猝睡症患者生活品質受嗜睡症狀及猝倒嚴重度改變直接影響，同時受人類白血球抗原屬性及神經認知功能改變調節之假設模型，作為未來研究參考依據。並根據研究結果與限制，提出對臨床實務的應用與心理介入的建議，並對未來提升猝睡症患者生活品質相關研究提供建議。 / The current study aims to: (1) examine the change of eight domains of quality of life in narcoleptics within five years, (2) investigate the impact of the change of symptom severity on different dimension of quality of life, as well as the influence associated with the change of neuro-cognitive function. There were 168 participants recruited from a medical center in northern Taiwan. 85 of them completed the 5-year annual follow-up data collection. During the follow-ups, polysomnography (PSG), multiple sleep latency test (MSLT) and human leukocyte antigen (HLA) test were conducted. Computerized neuropsychological tests of Conners’ Continuous Performance Test- II (CPT-II) and Wisconsin Card Sorting Test (WCST) were also administered to obtain attention and executive function data. The short from-36 items of health related quality of life (SF-36), Stanford sleep inventory (SSI) and Epworth sleepiness scale were applied to assess quality of life and symptom severity. Descriptive statistics and hierarchical linear models were applied for data analysis. The main results were: 1. The quality of life was divided into physical and psychological domains. The physical domain kept relatively stable during the 5-year follow up as opposed to the psychological domain. In psychological domain, the vitality and psychological health showed increasing tendency overtime. However, the social function and role functioning-emotion increased during the first 3 years then declined afterward. 2. The symptom severity also showed a tendency corresponded to quadratic curve. The daytime sleepiness together with cataplexy severity reduced immediately after treatment but rose after the third year. 3. The variables of individual characteristics that showed significant impact on quality of life were disease duration and HLA type. The longer the duration, the better quality of life one had. Positive HLA typing seemed to be a protective factor on severity of sleepiness. It also predicted better treatment outcomes, but worsen the severity of cataplexy and treatment effects. 4. The symptom severity could be a good explanation as a variable of quality of life. The daytime sleepiness altered all domain of SF-36 expect body pain. Cataplexy affected only psychological domain of SF-36. 5. The neuro-cognitive function was also found to affect quality of life. Those who improved in attention and executive function test got greater improvement on SF-36 as well. The vigilance on CPT-II and conceptualized response on WCST had most significant impact. I proposed a model of change of quality of life in patients with narcolepsy based on the results obtained. Several suggestions were also proposed for clinical and psychological intervention for narcolepsy to improve their quality of life.