Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands.
Sleep-Wakecentre SEIN, Heemstede, The Netherlands.
Sleep. 2020 Aug 12;43(8). doi: 10.1093/sleep/zsaa044.
The classification of the central disorders of hypersomnolence has undergone multiple iterations in an attempt to capture biologically meaningful disease entities in the absence of known pathophysiology. Accumulating data suggests that further refinements may be necessary. At the 7th International Symposium on Narcolepsy, a group of clinician-scientists evaluated data in support of keeping or changing classifications, and as a result suggest several changes. First, idiopathic hypersomnia with long sleep durations appears to be an identifiable and meaningful disease subtype. Second, idiopathic hypersomnia without long sleep time and narcolepsy without cataplexy share substantial phenotypic overlap and cannot reliably be distinguished with current testing, and so combining them into a single disease entity seems warranted at present. Moving forward, it is critical to phenotype patients across a wide variety of clinical and biological features, to aid in future refinements of disease classification.
中枢性嗜睡症的分类经历了多次迭代,试图在缺乏已知病理生理学的情况下捕捉具有生物学意义的疾病实体。越来越多的数据表明,可能需要进一步的细化。在第七届国际嗜睡症研讨会上,一组临床科学家评估了支持保留或更改分类的数据,结果提出了一些更改建议。首先,长睡眠时间的特发性嗜睡症似乎是一种可识别和有意义的疾病亚型。其次,无长睡眠时间的特发性嗜睡症和无猝倒的嗜睡症有很大的表型重叠,目前的测试无法可靠地区分,因此将它们合并为一个单一的疾病实体似乎是合理的。向前推进,对具有广泛临床和生物学特征的患者进行表型分析至关重要,以帮助未来对疾病分类进行细化。