Elphick, H, Staniforth, T, Blackwell, J orcid.org/0000-0002-5878-8959 et al. (1 more author) (2017) Narcolepsy and Cataplexy – a practical approach to diagnosis and managing the impact of this chronic condition on children and their families. Paediatrics and Child Health, 27 (7). pp. 343-347. ISSN 1751-7222
Abstract
Narcolepsy is a relatively common neurological condition affecting the regulation of normal sleep/wake cycles leading to excessive daytime sleepiness (EDS). It is almost certainly under-recognised as it has a prevalence of 20–50 per 100,000 population and most cases have an onset in adolescence. Cataplexy (attacks of muscle weakness often precipitated by strong emotions) is a hallmark of this condition and represents the intrusion of REM sleep into wakefulness.
Narcolepsy is caused by destruction of hypocretin producing cells due to an autoimmune process often by an infective trigger. Hypocretin is found in the hypothalamus and plays a role in stabilisation of the transition between wake and sleep states. In establishing a diagnosis a comprehensive history to exclude other causes of EDS, including poor sleep habits, is essential. Primary sleep related conditions such as sleep apnoea should be excluded. Investigations for confirmation of the diagnosis include Actigraphy, Polysomnography (PSG), Multiple Sleep Latency Testing (MSLT) and CSF analysis. The symptoms of this debilitating condition can have a huge impact on a child's life and are often vastly underestimated. The impact of EDS on cognitive function is an important factor in difficulties at school, mood, quality of life and future career opportunities. Advances in understanding the pathophysiology have led to trials of novel treatment approaches. The aim of this article is to briefly summarise the recent advances in understanding and give an overview of this important condition for those who are involved in the care of a child with this disease.
Metadata
Item Type: | Article |
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Authors/Creators: |
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Copyright, Publisher and Additional Information: | © 2017 Elsevier Ltd. This is an author produced version of a paper published in Paediatrics and Child Health. Uploaded in accordance with the publisher's self-archiving policy. |
Keywords: | Narcolepsy; Cataplexy; Excessive Daytime Sleepiness; Hypocretin; Multiple Sleep Latency Test; Cognitive function |
Dates: |
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Institution: | The University of Leeds |
Academic Units: | The University of Leeds > Faculty of Medicine and Health (Leeds) > School of Psychology (Leeds) |
Depositing User: | Symplectic Publications |
Date Deposited: | 10 Mar 2017 15:46 |
Last Modified: | 05 Jul 2018 12:17 |
Published Version: | https://doi.org/10.1016/j.paed.2017.02.007 |
Status: | Published |
Publisher: | Elsevier |
Identification Number: | 10.1016/j.paed.2017.02.007 |
Open Archives Initiative ID (OAI ID): | oai:eprints.whiterose.ac.uk:113457 |