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目的对比早发型及晚发型发作性睡病患者的临床及电生理检测特点。方法发作性睡病者共105例,其中15岁以下起病的早发型患者63例,平均发病年龄9.7岁±3.1岁;15岁以上起病的晚发型患者42例,平均发病年龄22.8岁±9.3岁。对比分析其临床、多导生理记录仪及多次小睡睡眠潜伏时间试验(MSLT)结果。结果全部患者均有白天嗜睡。发作性猝倒在早发型患者中发生率为92%,高于晚发型患者的76%(P=0.023);两组患者睡瘫、入睡幻觉及夜间睡眠紊乱的发生率相近(P均>0.05)。MSLT检查发现早发型患者的平均睡眠潜伏期(4.5 m in±4.0 m in及7.0 m in±5.7 m in,P=0.018)和REM睡眠潜伏期(3.4 m in±3.2 m in及4.8 m in±2.2 m in,P=0.02)更短,REM睡眠次数更多(3.4±2.0及2.5±1.9,P=0.009),晚发型患者的AHI次数更多(9.2次±16.5次及1.9次±6.3次,P=0.009),睡眠呼吸障碍较严重。3例有家族史者均为早发型患者。结论早发型发作性睡病患者的嗜睡及猝倒症状及临床特点较晚发型患者典型,其发生可能与遗传因素的关系更密切。
Objective To compare the clinical and electrophysiological characteristics of early-onset and late-onset narcolepsy patients. Methods A total of 105 cases of narcolepsy, including 63 cases of early onset of onset less than 15 years old, with an average age of 9.7 ± 3.1 years; 42 cases of late onset type of onset more than 15 years of age, the average age of onset of 22.8 ± 9.3 years old. Comparative analysis of its clinical, multi-channel physiological recorder and multiple sleep latency test (MSLT) results. Results All patients had daytime drowsiness. The incidence of catastrophe cataplasia was 92% in early-onset patients, which was higher than 76% in late-onset patients (P = 0.023). The incidence of paralysis, hallucination and nocturnal sleep disturbances were similar in both groups (P> 0.05 ). The mean sleep latency (4.5 m in ± 4.0 m in and 7.0 m in ± 5.7 m in, P = 0.018) and REM sleep latency (3.4 m in ± 3.2 m in and 4.8 m in ± 2.2 m in patients with early-onset MSLT) (P = 0.009). The number of AHI was higher in late-onset patients (9.2 ± 16.5 times and 1.9 ± 6.3 times, P = 0.009), sleep disordered breathing is more serious. Three patients with family history were early-onset patients. Conclusions Patients with early-onset narcolepsy may have narcolepsy and cataplasia symptoms and their clinical features are more typical of later-onset patients. Their occurrence may be more closely related to genetic factors.