OBJECTIVE Narcolepsy management usually requires lifelong pharmacotherapy. However, we know little about adherence to prescribed treatment in narcolepsy. We assessed adherence to wakefulness-promoting agents in narcolepsy patients. PATIENTS AND METHODS We retrospectively assessed adherence to wakefulness promoting medication in patients with narcolepsy using the Medicines Possession Ratio (MPR). Three levels of adherence were defined: poor (≤50%), intermediate (51-79%), and good (≥80%). Refractory daytime sleepiness was defined as an Epworth sleepiness scale (ESS) score >12 despite trialling at least three wakefulness-promoting agents. We compared demographic and clinical factors, and prescribed medications between patients, stratified by levels of adherence, as well as by presence or not of refractory sleepiness. RESULTS We included 116 patients with narcolepsy (54.3% female, mean age 39.4 (±14) years). In sum, 93 (80.2%) patients had a diagnosis of narcolepsy type 1 (NT1), and 23 (19.8%) of type 2 (NT2). Suboptimal symptom control was common: 39.8% had refractory sleepiness, and 47.3% of NT1 patients had persistent cataplexy. Good adherence was seen in only 55.2% of patients, while 12.9% were intermediately and 31.9% poorly adherent. Patients with poor adherence were more likely to have a diagnosis of NT2, but adherence did not vary according to gender, age, the presence of psychiatric co-morbidity, or the presence of apparent intractable symptoms. Levels of good adherence to therapy were no better in patients with refractory sleepiness than in those with satisfactory symptom control (56.5% vs 54.3%; p = 0.81). CONCLUSION Suboptimal adherence to prescribed therapy is common in narcolepsy patients, including those with apparent intractable symptoms, and particularly in patients with NT2.

中文翻译：

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对嗜睡症患者坚持清醒促进药物治疗。

目的发作性睡病治疗通常需要终身药物治疗。但是，我们对发作性睡病坚持处方治疗知之甚少。我们评估了发作性睡病患者对清醒促进剂的依从性。患者与方法我们使用药物占有率（MPR）回顾性评估了发作性睡病患者对清醒促进药物的依从性。定义了三个级别的依从性：差（≤50％），中级（51-79％）和良好（≥80％）。尽管试用了至少三种觉醒促进剂，但难治性白天嗜睡定义为Epworth嗜睡量表（ESS）得分> 12。我们比较了人口统计学和临床​​因素，以及患者之间的处方药，按依从性水平以及是否存在难治性嗜睡进行分层。结果我们纳入了116例发作性睡病患者（女性54.3％，平均年龄39.4（±14）岁）。总之，有93位（80.2％）患者被诊断为1型发作性睡病（NT1），而23位（29.8％）诊断为发作性睡病。症状控制不佳很常见：39.8％的患者有难治性嗜睡，NT1患者中的47.3％患有持续性瘫痪。仅55.2％的患者观察到良好的依从性，而中度依从性为12.9％，依从性差为31.9％。依从性差的患者更有可能被诊断为NT2，但是依从性并未因性别，年龄，精神病合并症的存在或明显的顽固性症状而异。难治性嗜睡的患者对治疗的良好依从性水平没有症状控制满意的患者（56.5％vs 54.3％； p = 0.81）。