Over the next 10 years, two different groups proposed the terms “essential narcolepsy” and “NREM narcolepsy for this category of patients while Roth continued to study them. who, in 1966 wrote: “Subjects with excessive daytime sleepiness but no cataplexy, sleep paralysis or sleep onset REM periods do not have narcolepsy and should be relegated to another diagnostic category”. By the mid 1960’s Roth’s work was confirmed by other researchers including Dement et al. In 1960, Vogel showed that narcoleptic patients fall directly into REM sleep, paving the way to a more accurate distinction between these different forms of hypersomnolence. It was during this time that Roth started to realise that patients with hypersomnia but without the classic clinical features of narcolepsy and without any other explanation for their symptoms were suffering from an independent clinical entity. One year after the description of sleep drunkenness in his 1956 paper Roth published a book entitled “Narcolepsy and hypersomnia, from the aspect of physiology of sleep”. Roth’s early work, dating back to the early 1950’s included a number of studies where he recognised and recorded the clinical differences between narcolepsy and hypersomnia. The identification of idiopathic hypersomnia started with the first description of sleep drunkenness by Bedrich Roth in Prague in 1956 and culminating in the name idiopathic hypersomnia and the initial description of the condition with two forms, polysymptomatic and monosymptomatic by Bedrich Roth in 1976. Roth also noted that N2 was more similar to what he described as monosymptomatic hypersomnia (referred to in the ICSD-2 as idiopathic hypersomnia without long sleep ) than it is to N1. With the discovery of hypocretin/orexin deficiency being unique in narcolepsy with cataplexy (now known as narcolepsy type 1 ) we now know that Roth’s very early observations were accurate in that N1 and idiopathic hypersomnia are separate clinical entities. Research into narcolepsy has come a long way since Roth’s early narcolepsy epidemiology studies. Consequently, a lot of the work that has been done has not advanced the epidemiological, etiological or pathophysiological understanding of idiopathic hypersomnia thus our knowledge has not moved on much further from what we have learnt from Bedrich Roth’s original work. One issue is that the MSLT was considered the ‘gold standard’ with regards to diagnosing idiopathic hypersomnia and narcolepsy, however, research has shown not only it’s inadequacy in diagnosing idiopathic hypersomnia and narcolepsy without cataplexy (now known as narcolepsy type 2 ) but also its inability to accurately distinguish N2 from idiopathic hypersomnia. *You may find this interesting if you are treating patients with Narcolepsy Type 2 (without cataplexy) or are a patient yourself.Ĭompared to the advances in narcolepsy research there has unfortunately not been a lot of meaningful progress made with idiopathic hypersomnia. Drawn from nearly 60 references, including at least 40 peer reviewed studies on Idiopathic Hypersomnia and Narcolepsy that span more than 6 decades as well as numerous personal conversations with the world's leading Idiopathic Hypersomnia researchers. A comprehensive review of how we have gone from the identification of Idiopathic Hypersomnia to where we are now.
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