Idiopathic hypersomnia: A study of 77 cases

Idiopathic hypersomnia: A study of 77 cases
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DOI:
10.1093/sleep/30.10.1274
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发表时间:
2007-10-01
期刊:
影响因子:
5.6
通讯作者:
Shneerson, John M.
Shneerson, John M.
中科院分区:
医学2区
文献类型:
--
作者:
Anderson, Kirstie N.;Pilsworth, Samantha;Shneerson, John M.

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研究目的:回顾特发性睡眠过度的临床和多导睡眠图特征以及对治疗的长期反应。设置:英国剑桥Papworth医院的呼吸支持和睡眠中心。患者和设计:对6000多例睡眠障碍患者的大型数据库进行了回顾。对77例特发性睡眠过度患者的临床和多导睡眠图特征进行了回顾性研究。与一组相似的发作性睡病患者进行比较。对药物治疗的反应进行了评估,在61例患者的平均随访3.8 years. Measures和结果:特发性嗜睡症的发病率为60%。与一组相似的发作性睡病患者相比,特发性睡眠过度患者更有可能有长时间的不清醒的日间小睡,阳性家族史,慢波睡眠增加,以及多次睡眠潜伏期测试的睡眠潜伏期更长。多次睡眠潜伏期测试的结果对预测疾病严重程度或治疗反应没有帮助。临床特征是异质性和可变的严重程度。大多数特发性嗜睡症患者的症状多年来保持稳定,但11%的患者自发缓解,这在嗜睡症中从未见过。三分之二的特发性嗜睡患者有持续改善白天嗜睡与药物治疗,虽然三分之一需要高剂量或组合的drugs.Conclusions:特发性嗜睡具有特征性的临床和多导睡眠图功能,但延长潜伏期的多次睡眠潜伏期测试提出了怀疑,在目前的诊断标准,这项测试的有效性。这种疾病通常对治疗反应良好,并且相当多的少数患者似乎自发改善。
Study Objectives: To review the clinical and polysomnographic characteristics of idiopathic hypersomnia as well as the long-term response to treatment.Setting: The Respiratory Support and Sleep Centre at Papworth Hospital, Cambridge, UK.Patients and Design: A large database of more than 6000 patients with sleep disorders was reviewed. A retrospective study of the clinical and polysomnographic characteristics of 77 patients with idiopathic hypersomnia was performed. Comparison with a similar group of patients with narcolepsy was performed. The response to drug treatment was assessed in 61 patients over a mean follow-up of 3.8 years.Measurements and Results: Idiopathic hypersomnia was 60% as prevalent as narcolepsy. Comparison with a similar group of patients with narcolepsy showed that those with idiopathic hypersomnia were more likely to have prolonged unrefreshing daytime naps, a positive family history, increased slow-wave sleep, and a longer sleep latency on the Multiple Sleep Latency Test. The results of the Multiple Sleep Latency Test were not helpful in predicting disease severity or treatment response. The clinical features were heterogeneous and of variable severity. The majority of patients with idiopathic hypersomnia had symptoms that remained stable over many years, but 11% had spontaneous remission, which was never seen in narcolepsy. Two thirds of patients with idiopathic hypersomnolence had a sustained improvement in daytime somnolence with medication, although a third needed high doses or combinations of drugs.Conclusions: Idiopathic hypersomnolence has characteristic clinical and polysomnographic features but the prolonged latency on the Multiple Sleep Latency Test raises doubt about the validity of this test within the current diagnostic; criteria. The disease often responds well to treatment and a substantial minority of patients appear to spontaneously improve.