A practical guide to the therapy of narcolepsy and hypersomnia syndromes.

A practical guide to the therapy of narcolepsy and hypersomnia syndromes.
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DOI:
10.1007/s13311-012-0150-9
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发表时间:
2012-10
期刊:
影响因子:
5.7
通讯作者:
Mignot, Emmanuel J. M.
Mignot, Emmanuel J. M.
中科院分区:
医学2区
文献类型:
--
作者:
Mignot, Emmanuel J. M.

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发作性睡病和其他与白天过度嗜睡相关的症状可能很难治疗。新的分类现在将发作性睡病/下丘脑肌素缺乏症(也称为1型发作性睡病)与其他原因不明的睡眠症区分开来。发作性睡病是一种终生疾病,具有完善的诊断程序和病因。克莱因-莱文综合征是一种与认知和行为异常相关的周期性睡眠症,也被认为是一个独立的实体,有不同的治疗方案。非降克素相关的嗜睡综合征是排除的诊断。只有在消除了睡眠剥夺、睡眠呼吸暂停、夜间睡眠障碍和作为白天嗜睡的主要原因的精神合并症之后,才能做出这些诊断。发作性睡病/下丘脑肌醇缺乏症的治疗有很好的编纂,包括使用羟丁酸钠、兴奋剂和/或抗抑郁药的药物治疗,以及行为改变。这些疗法几乎总是需要的,风险与收益的比率是明确的,特别是在儿童中。为了优化使用,需要详细了解每种化合物的药理特性。对其他睡眠过多综合征的治疗更具挑战性,也更不规范。治疗最好是保守的(如莫达非尼、托莫西汀、行为改变),但可能必须更具侵略性(高剂量兴奋剂、羟丁酸钠等)。在逐案、经验性审判的基础上。由于在这些情况下病因和演变尚不清楚,随着时间的推移,重要的是挑战诊断和治疗,记住耐受的可能性和兴奋剂成瘾的发展。Kleine-Levin综合征通常最好不治疗,尽管锂可以被认为是发作频繁的严重病例。指南是基于文献和作者的个人经验而提供的。本文的在线版本(doi:10.1007/s13311-0120150-9)包含补充材料,授权用户可以使用。
Narcolepsy and other syndromes associated with excessive daytime sleepiness can be challenging to treat. New classifications now distinguish narcolepsy/hypocretin deficiency (also called type 1 narcolepsy), a lifelong disorder with well-established diagnostic procedures and etiology, from other syndromes with hypersomnolence of unknown causes. Klein-Levin Syndrome, a periodic hypersomnia associated with cognitive and behavioral abnormalities, is also considered a separate entity with separate therapeutic protocols. Non hypocretin-related hypersomnia syndromes are diagnoses of exclusion. These diagnoses are only made after eliminating sleep deprivation, sleep apnea, disturbed nocturnal sleep, and psychiatric comorbidities as the primary cause of daytime sleepiness. The treatment of narcolepsy/hypocretin deficiency is well-codified, and involves pharmacotherapies using sodium oxybate, stimulants, and/or antidepressants, plus behavioral modifications. These therapies are almost always needed, and the risk-to-benefit ratio is clear, notably in children. Detailed knowledge of the pharmacological profile of each compound is needed to optimize use. Treatment for other syndromes with hypersomnolence is more challenging and less codified. Preferably, therapy should be conservative (such as modafinil, atomoxetine, behavioral modifications), but it may have to be more aggressive (high-dose stimulants, sodium oxybate, etc.) on a case-by-case, empirical trial basis. As cause and evolution are unknown in these conditions, it is important to challenge diagnosis and therapy over time, keeping in mind the possibility of tolerance and the development of stimulant addiction. Kleine-Levin Syndrome is usually best left untreated, although lithium can be considered in severe cases with frequent episodes. Guidelines are provided based on the literature and personal experience of the author. The online version of this article (doi:10.1007/s13311-012-0150-9) contains supplementary material, which is available to authorized users.
DOI: 10.1345/aph.1l523
发表时间: 2009-06-01
影响因子: 2.9
作者:
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DOI: 10.1081/clt-120019128
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发表时间: 2008-04-01
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发表时间: 2011-01-01
影响因子: 2.5
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DOI: 10.1212/wnl.49.2.444
发表时间: 1997-08-01
期刊: NEUROLOGY
影响因子: 9.9
作者:
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