Sleep problems in Dravet syndrome: a modifiable comorbidity
Open accessAbstract
Aim: Many children with severe developmental and epileptic encephalopathies experience significant sleep disturbance, causing major disruption to the family's quality of life. We aimed to determine the frequency and nature of sleep problems in individuals with Dravet syndrome. Methods: The Sleep Disturbance Scale for Children and a seizure questionnaire were distributed to the parents/guardians of 96 patients with Dravet syndrome. Sixteen patients had two nights of home oximetry. Results: Fifty‐seven out of 96 questionnaires were completed. Forty‐three out of 57 (75%) individuals had sleep problems. Twenty‐five out of 57 (44%) individuals had an abnormal total sleep score, with difficulty initiating and maintaining sleep (22 out of 57, 39%), sleep–wake transition disorders (20 out of 57, 35%), and sleep breathing disorders (19 out of 57, 33%). Twenty‐two out of 57 (39%) individuals took medication to assist sleep, predominantly melatonin ( n =14). Thirty out of 57 (53%) recently had nocturnal seizures. Overnight oximetry showed 14 out of 16 (88%) had a higher oxygen desaturation index (>3%), and six out of 16 (38%) had higher mean pulse rates than normative values. Home oximetry was normal or inconclusive in all patients. Interpretation: Seventy‐five per cent of individuals with Dravet syndrome had sleep problems, highlighting the importance of routinely assessing sleep and initiating appropriate behavioural and pharmacological interventions to improve the patient and family's quality of life. A high oxygen desaturation index and mean pulse rates on pulse oximetry may reflect unrecognized nocturnal seizures. What this paper adds: More than 70% of patients with Dravet syndrome have sleep problems. Difficulty initiating and maintaining sleep was most common, particularly in those older than 20 years. Second most common were sleep–wake transition disorders, affecting more than 50% of those younger than 5 years. Sleep breathing disorders were a frequent problem across all age groups. Oximetry was not diagnostic of sleep‐disordered breathing or obvious seizures.
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DEEs often include speech, sleep, respiratory and gastrointestinal problems.
“speech 154 , sleep 155 , respiratory and gastrointestinal problems.”
From Developmental and epileptic encephalopathies (2024) · cites this paper as [155]
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Cites
Bruni, O., Ottaviano, S., Guidetti, V., ROMOLI, M., Innocenzi, M., Cortesi, F., & Giannotti, F. (1996) · Journal of Sleep Research · doi:10.1111/j.1365-2869.1996.00251.x · Cited by 1,385 · see citing works
De Novo Mutations in the Sodium-Channel Gene SCN1A Cause Severe Myoclonic Epilepsy of Infancy
Claes, L., Del-Favero, J., Ceulemans, B., Lagae, L., Van Broeckhoven, C., & De Jonghe, P. (2001) · The American Journal of Human Genetics · doi:10.1086/320609 · Cited by 1,292 · see citing works
Ryvlin, P., Nashef, L. A. M., Lhatoo, S. D., Bateman, L. M., Bird, J., Bleasel, A., Boon, P., Crespel, A., Dworetzky, B. A., Høgenhaven, H., et al. (2013) · The Lancet Neurology · doi:10.1016/s1474-4422(13)70214-x · Cited by 1,137 · see citing works
Krakowiak, P., Goodlin‐Jones, B. L., Hertz‐Picciotto, I., Croen, L., & Hansen, R. L. (2008) · Journal of Sleep Research · doi:10.1111/j.1365-2869.2008.00650.x · Cited by 589 · see citing works
Nocturnal Pulse Oximetry as an Abbreviated Testing Modality for Pediatric Obstructive Sleep Apnea
Brouillette, R. T., Morielli, A., Leimanis, A., Waters, K. A., Luciano, R., & Ducharme, F. M. (2000) · PEDIATRICS · doi:10.1542/peds.105.2.405 · Cited by 575 · see citing works
Skluzacek, J. V., Watts, K. P., Parsy, O., Wical, B., & Camfield, P. (2011) · Epilepsia · doi:10.1111/j.1528-1167.2011.03012.x · Cited by 170 · see citing works
The Relationship Between Sleep and Epilepsy
Kotagal, P., & Yardi, N. (2008) · Seminars in Pediatric Neurology · doi:10.1016/j.spen.2008.03.007 · Cited by 155 · see citing works
Scholle, S., Wiater, A. H., & Scholle, H. C. (2011) · Sleep Medicine · doi:10.1016/j.sleep.2011.05.006 · Cited by 150 · see citing works
Obstructive Sleep Apnea and Respiratory Complications Associated with Vagus Nerve Stimulators
Parhizgar, F., NUGENT, K. M., & Raj, R. (2011) · Journal of Clinical Sleep Medicine · doi:10.5664/jcsm.1204 · Cited by 129 · see citing works
Alanis-Guevara, I., Peña, E., Corona, T., López-Ayala, T., López-Meza, E. G., & Lopez-Gómez, M. (2005) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2005.06.010 · Cited by 122 · see citing works
Sleep impairment and reduced interneuron excitability in a mouse model of Dravet Syndrome
Kalume, F. K., Oakley, J. C., Westenbroek, R. E., Gile, J. J., de la Iglesia, H. O., Scheuer, T., & Catterall, W. A. (2015) · Neurobiology of Disease · doi:10.1016/j.nbd.2015.02.016 · Cited by 109 · see citing works
Electrical and autonomic cardiac function in patients with Dravet syndrome
Delogu, A. B., Spinelli, A., Battaglia, D., Dravet, C., Nisco, A. D., Saracino, A., Romagnoli, C., Lanza, G. A., & Crea, F. (2011) · Epilepsia · doi:10.1111/j.1528-1167.2011.03003.x · Cited by 92 · see citing works
Melatonin improves sleep in children with epilepsy: a randomized, double-blind, crossover study
Jain, S. V., Horn, P. S., Simakajornboon, N., Beebe, D. W., Holland, K. D., Byars, A. W., & Glauser, T. A. (2015) · Sleep Medicine · doi:10.1016/j.sleep.2015.01.005 · Cited by 87 · see citing works
Infant and toddler sleep in Australia and New Zealand
Teng, A. Y., Bartle, A., Sadeh, A., & Mindell, J. A. A. (2011) · Journal of Paediatrics and Child Health · doi:10.1111/j.1440-1754.2011.02251.x · Cited by 85 · see citing works
Papale, L. A., Makinson, C. D., Ehlen, J. C., Tufik, S., Decker, M. J., Paul, K. N., & Escayg, A. (2013) · Epilepsia · doi:10.1111/epi.12060 · Cited by 64 · see citing works
Autism and behavior in adult patients with Dravet syndrome (DS)
Berkvens, J. J. L., Veugen, I., Veendrick-Meekes, M. J. B. M., Snoeijen-Schouwenaars, F. M., Schelhaas, H. J., Willemsen, M. H., Tan, I. Y., & Aldenkamp, A. P. (2015) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2015.04.057 · Cited by 60 · see citing works
Determinants of sleep disturbances in Rett syndrome: Novel findings in relation to genotype
Boban, S., Wong, K., Epstein, A., et al. (2016) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.37784 · Cited by 56 · see citing works
Subjective sleep disturbances in children with partial epilepsy and their effects on quality of life
Gutter, T., Brouwer, O. F., & de Weerd, A. W. (2013) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2013.06.022 · Cited by 52 · see citing works
Evaluation of sleep disturbances in children with epilepsy: A questionnaire-based case–control study
Chan, B., Yau, E. K., Ng, S.-F., Chan, Y. C., Lee, Q. U., & Chan, K. Y. (2011) · Epilepsy & Behavior · doi:10.1016/j.yebeh.2011.05.007 · Cited by 42 · see citing works
Sleep Abnormalities in Children With Dravet Syndrome
Dhamija, R., Erickson, M. K., Louis, E. K. S., Wirrell, E. C., & Kotagal, S. (2014) · Pediatric Neurology · doi:10.1016/j.pediatrneurol.2014.01.017 · Cited by 36 · see citing works
Nocturnal seizures and the effects of anticonvulsants on sleep
Bazil, C. W. (2008) · Current Neurology and Neuroscience Reports · doi:10.1007/s11910-008-0024-z · Cited by 32 · see citing works
Children's Sleep Disturbance Scale in Differentiating Neurological Disorders
Cohen, R., Halevy, A., & Shuper, A. (2013) · Pediatric Neurology · doi:10.1016/j.pediatrneurol.2013.06.010 · Cited by 28 · see citing works
Melatonin as add-on treatment for epilepsy
Brigo, F., & Felice, A. D. (2012) · Cochrane Database of Systematic Reviews · doi:10.1002/14651858.cd006967.pub2 · Cited by 21 · see citing works
Oximetry for suspected obstructive sleep apnea-Does removal of awake data affect the result?
Nixon, G. M., Davey, M. J., Weichard, A. J., et al. (2016) · Pediatric Pulmonology · doi:10.1002/ppul.23486 · Cited by 8 · see citing works
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Research works printed but not linked: 1
- [1]Dravet C, Bureau M, Oguni H, Cokar O, Guerrini R. In Bureau M, Genton P, Dravet C, et al., editors. Epileptic Syndromes in Infancy, Chidhood and Adolescence (5th edition). Montrouge: John Libbey Eurotext, 2012: 125-56.
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Developmental and epileptic encephalopathies
Scheffer, I. E., Zuberi, S., Mefford, H. C., Guerrini, R., & McTague, A. (2024) · Nature Reviews Disease Primers · doi:10.1038/s41572-024-00546-6 · Cited by 128 · see citing works
Self-citationPreclinical Animal Models for Dravet Syndrome: Seizure Phenotypes, Comorbidities and Drug Screening
Griffin, A., Hamling, K. R., Hong, S., Anvar, M., Lee, L. P., & Baraban, S. C. (2018) · Frontiers in Pharmacology · doi:10.3389/fphar.2018.00573 · Cited by 103 · see citing works
Palmer, E. E., Howell, K., & Scheffer, I. E. (2021) · Neurotherapeutics · doi:10.1007/s13311-021-01133-3 · Cited by 53 · see citing works
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- Authors
- Licheni, S. H., Mcmahon, J. M., Schneider, A. L., Davey, M. J., & Scheffer, I. E.
- Affiliations
- The University of Melbourne (AU)
- Monash Children’s Hospital (AU)
- Royal Children's Hospital (AU)
- Corresponding author
- Scheffer, Ingrid E
- Venue
- Developmental Medicine & Child Neurology
- Publisher
- Wiley
- Published
- 2017-11-07
- Volume
- 60
- Issue
- 2
- Pages
- 192-198
- ISSN
- 0012-1622 (ISSN-L)
- 1469-8749
- OpenAlex
- W2767422484
- Cited by
- 85
- References
- 24
- Citations by year
- 2026: 15 · 2025: 11 · 2024: 14 · 2023: 7 · 2022: 13 · 2021: 10 · 2020: 6 · 2019: 2 · 2018: 6 · 2017: 1
- FWCI
- 5.30
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Developmental Medicine & Child Neurology (publishedVersion)
- PubMed (publishedVersion)
- Minerva Access (University of Melbourne) (submittedVersion)
- Funders
- National Health and Medical Research Council
- Topics
- Social Sciences › Psychology › Experimental and Cognitive Psychology › Sleep and related disorders
- Health Sciences › Medicine › Psychiatry and Mental health › Epilepsy research and treatment
- Life Sciences › Neuroscience › Endocrine and Autonomic Systems › Circadian rhythm and melatonin
- Keywords
- Dravet syndrome, sleep disorders, sleep-wake transition disorders, sleep-disordered breathing, nocturnal seizures, oxygen desaturation index, pulse oximetry, melatonin, sleep initiation and maintenance, developmental and epileptic encephalopathy, comorbidities, quality of life
- MeSH terms
- Adolescent, Adult, Child, Child, Preschool, Epilepsies, Myoclonic, Female, Humans, Male, Oximetry, Quality of Life, Surveys and Questionnaires, Sleep Wake Disorders, Comorbidity, Young Adult
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