Rye D B, Dihenia B, Weissman J D, Epstein C M, Bliwise D L
Department of Neurology, Emory University School of Medicine, Atlanta, GA, USA.
Neurology. 1998 Feb;50(2):459-65. doi: 10.1212/wnl.50.2.459.
To advance understanding of the clinical spectra of narcolepsy, we retrospectively reviewed the histories and clinical and polysomnographic features of 41 consecutive patients in whom this diagnosis was established in our center over 3 years. A total of 51% presented after the age of 40 years. Among the older patients, three subpopulations were noted: 1) narcolepsy/cataplexy with presentation delayed because of mild disease severity or misdiagnosis; 2) narcolepsy/cataplexy with diagnosis delayed until late-life expression of cataplexy; and 3) narcolepsy lacking cataplexy with later-life onset of excessive daytime sleepiness. Clinical, polysomnographic, and multiple sleep latency test assessments of rapid eye movement sleep dyscontrol and sleepiness were unrelated to age. This analysis identified older patients lacking cataplexy as the least severely affected narcoleptic subgroup. Narcolepsy, a continuum of phenotypes and severities that masks its recognition, should be considered in the differential diagnosis of sleepiness or transient loss of muscle tone in older patients.
为了加深对发作性睡病临床谱的理解,我们回顾性分析了连续41例在我们中心3年内确诊的患者的病史、临床及多导睡眠图特征。共有51%的患者在40岁以后发病。在老年患者中,发现了三个亚组:1)发作性睡病/猝倒症,因疾病严重程度较轻或误诊导致发病延迟;2)发作性睡病/猝倒症,诊断延迟至晚年出现猝倒症状;3)无猝倒的发作性睡病,晚年出现白天过度嗜睡。对快速眼动睡眠失调和嗜睡的临床、多导睡眠图及多次睡眠潜伏期测试评估与年龄无关。该分析确定无猝倒的老年患者是发作性睡病患者中受影响最轻的亚组。发作性睡病是一种表现型和严重程度连续的疾病,容易被忽视,在老年患者出现嗜睡或短暂肌张力丧失的鉴别诊断中应考虑该病。