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Journal of Neurodevelopmental Disorders

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Open Access 01-12-2018 | Research

The feasibility of using actigraphy to characterize sleep in Rett syndrome

Authors: Alyssa M. Merbler, Breanne J. Byiers, John J. Garcia, Timothy J. Feyma, Frank J. Symons

Published in: Journal of Neurodevelopmental Disorders | Issue 1/2018

Abstract

Background

Rett syndrome (RTT) is a neurodevelopmental disorder primarily caused by mutations in the MECP2 gene. Sleep problems are reported by the majority of caregivers of individuals with RTT.

Methods

The present study aimed to replicate and extend previous work about the feasibility of measuring sleep with an actigraph device in a sample of girls with clinically diagnosed RTT (N = 13, mean age = 9 years, 5 months). Participants wore an actigraph device day and night for seven consecutive days. Materials also included a parent-completed sleep diary to measure bedtime, duration of nighttime sleep, and daytime sleep, and the Child Sleep Habit’s Questionnaire (CSHQ).

Results

The means for the sample as measured by actigraphy were 492.3 min (SD = 47.3) of total night sleep (TNS), 76.0% (SD = 6.7) sleep efficiency, 86.0 min (SD = 34.2) of wake after sleep onset, and 46.1 min (50.8) of sleep when parents reported a nap occurring. Parents reported 589.7 min (SD = 53.6) of TNS, 15.9 min (SD = 12.0) of WASO, and 93.6 min (SD = 66.8) of daytime sleep according to sleep diaries, with all parents reporting at least one nap during the week. Relations were found between sleep characteristics and seizure status and CSHQ total scores. No age-related changes were observed for any sleep characteristic, regardless of collection method. Five of nine participants above the cutoff score on the CSHQ indicate the need for further evaluation for a sleep disorder.

Conclusions

Overall, actigraphy was feasible in this community-based sample of girls with RTT. The results replicated some aspects of previous studies of sleep in RTT (e.g., no age-related changes in total nighttime sleep or efficiency). Some participants met the American Academy of Sleep Medicine guidelines for recommended total sleep time, with others showing too much or too little sleep. Each of the three methods for describing sleep presented its own advantages and challenges. Future work should be prospectively designed, validate the use of actigraphy in this population, and include a typically developing comparison sample to improve the precision of our understanding of sleep in RTT.

Hagberg B. Clinical manifestations and stages of Rett syndrome. Ment Retard Dev Disabil Res Rev. 2002;8:61–5.CrossRefPubMed

Neul JL. The relationship of Rett syndrome and MECP2 disorders to autism. Dialogues Clin Neurosci. 2012;14:253–62.PubMedPubMedCentral

Amir RE, Van den Veyver IB, Wan M, Tran CQ, Francke U, Zoghbi HY. Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2. Nat Genet. 1999;23:185–8.CrossRefPubMed

Symons FJ, Byiers B, Hoch J, Dimian A, Barney C, Feyma T, et al. Infrared thermal analysis and individual differences in skin temperature asymmetry in Rett syndrome. Pediatr Neurol. 2015;53:169–72.CrossRefPubMedPubMedCentral

Neul JL, Kaufmann WE, Glaze DG, Christodoulou J, Clarke AJ, Bahi-Buisson N, et al. Rett syndrome: revised diagnostic criteria and nomenclature. Ann Neurol. 2010;68:944–50. https://doi.org/10.1002/ana.22124.CrossRefPubMedPubMedCentral

Young D, Nagarajan L, de Klerk N, Jacoby P, Ellaway C, Leonard H. Sleep problems in Rett syndrome. Brain and Development. 2007;29:609–16.CrossRefPubMedPubMedCentral

Ellaway C, Peat J, Leonard H, Christodoulou J. Sleep dysfunction in Rett syndrome: lack of age related decrease in sleep duration. Brain and Development. 2001;23:S101–3.CrossRefPubMed

Boban S, Wong K, Epstein A, Anderson B, Murphy N, Downs J, et al. Determinants of sleep disturbances in Rett syndrome: novel findings in relation to genotype. Am J Med Genet Part A. 2016;170(9): 2292–300.

Miyamoto A, Oki J, Takahashi S, Okuno A. Serum melatonin kinetics and long-term melatonin treatment for sleep disorders in Rett syndrome. Brain and Development. 1999;21:59–62.CrossRefPubMed

10.

Tsuchiya Y, Minami Y, Umemura Y, Watanabe H, Ono D, Nakamura W, et al. Disruption of MeCP2 attenuates circadian rhythm in CRISPR/Cas9-based Rett syndrome model mouse. Genes Cells. 2015;20:992–1005.CrossRefPubMed

11.

Li Q, Loh DH, Kudo T, Truong D, Derakhshesh M, Kaswan ZM, et al. Circadian rhythm disruption in a mouse model of Rett syndrome circadian disruption in RTT. Neurobiol Dis. 2015;77:155–64.CrossRefPubMed

12.

McDougall A, Kerr AM, Espie C a. Sleep disturbance in children with rett syndrome: a qualitative investigation of the parental experience. J Appl Res Intellect Disabil. 2005;18:201–15.CrossRef

13.

Dewald JF, Meijer AM, Oort FJ, Kerkhof GA, Bögels SM. The influence of sleep quality, sleep duration and sleepiness on school performance in children and adolescents: a meta-analytic review. Sleep Med Rev. 2010;14:179–89.CrossRefPubMed

14.

Sadeh A, Gruber R, Raviv A. Sleep, neurobehavioral functioning, and behavior problems in school-age children. Child Dev. 2002;73:405–17.CrossRefPubMed

15.

Owens JA, Spirito A, McGuinn M. The Children’s Sleep Habits Questionnaire (CSHQ): psychometric properties of a survey instrument for school-aged children. Sleep. 2000;23:1043–51. https://doi.org/10.1111/j.1469-8749.2001.tb00204.x.CrossRefPubMed

16.

Souders MC, Mason TB a, Valladares O, Bucan M, Levy SE, Mandell DS, et al. Sleep behaviors and sleep quality in children with autism spectrum disorders. Sleep. 2009;32:1566–78.CrossRefPubMedPubMedCentral

17.

Goldman SE, Bichell TJ, Surdyka K, Malow BA. Sleep in children and adolescents with Angelman syndrome: association with parent sleep and stress. J Intellect Disabil Res. 2012;56:600–8.CrossRefPubMed

18.

Annaz D, Hill CM, Ashworth A, Holley S, Karmiloff-Smith A. Characterisation of sleep problems in children with Williams syndrome. Res Dev Disabil. 2010;32:164–9.CrossRef

19.

McArthur a J, Budden SS. Sleep dysfunction in Rett syndrome: a trial of exogenous melatonin treatment. Dev Med Child Neurol. 1998;40:186–92.CrossRefPubMed

20.

Piazza CC, Fisher W, Moser H. Behavioral treatment of sleep dysfunction in patients with the Rett syndrome. Brain and Development. 1991;13:232–7. https://doi.org/10.1016/S0387-7604(12)80055-6.CrossRefPubMed

21.

Piazza CC, Fisher W, Kiesewetter K, Bowman L, Moser H. Aberrant sleep patterns in children with the Rett syndrome. Brain and Development. 1990;12:488–93. https://doi.org/10.1016/S0387-7604(12)80213-0.CrossRefPubMed

22.

Marcus CL, Carroll JL, McColley S a, Loughlin GM, Curtis S, Pyzik P, et al. Polysomnographic characteristics of patients with Rett syndrome. J Pediatr. 1994;125:218–24.CrossRefPubMed

23.

Nomura Y, Segawa M, Hasegawa M. Rett syndrome––clinical studies and pathophysiological consideration. Brain and Development. 1984;6:475–86. https://doi.org/10.1016/S0387-7604(84)80030-3.CrossRefPubMed

24.

Glaze DG, Frost JD, Zoghbi HY, Percy AK. Rett’s syndrome: characterization of respiratory patterns and sleep. Ann Neurol. 1987;21:377–82. https://doi.org/10.1002/ana.410210410.CrossRefPubMed

25.

Wong K, Leonard H, Jacoby P, Ellaway C, Downs J. The trajectories of sleep disturbances in Rett syndrome. J Sleep Res. 2015;24:223–33. https://doi.org/10.1111/jsr.12240.CrossRefPubMed

26.

Sadeh A. The role and validity of actigraphy in sleep medicine: an update. Sleep Med Rev. 2011;15:259–67. https://doi.org/10.1016/j.smrv.2010.10.001.CrossRefPubMed

27.

Wasdell MB, Jan JE, Bomben MM, Freeman RD, Rietveld WJ, Tai J, et al. A randomized, placebo-controlled trial of controlled release melatonin treatment of delayed sleep phase syndrome and impaired sleep maintenance in children with neurodevelopmental disabilities. J Pineal Res. 2008;44:57–64.PubMed

28.

Allen KD, Kuhn BR, DeHaai KA, Wallace DP. Evaluation of a behavioral treatment package to reduce sleep problems in children with Angelman Syndrome. Res Dev Disabil. 2013;34:676–86. https://doi.org/10.1016/j.ridd.2012.10.001.CrossRefPubMed

29.

Meltzer LJ, Montgomery-Downs HE, Insana SP, Walsh CM. Use of actigraphy for assessment in pediatric sleep research. Sleep Med Rev. 2012;16:463–75. https://doi.org/10.1016/j.smrv.2011.10.002.CrossRefPubMedPubMedCentral

30.

Temudo T, Oliveira P, Santos M, Dias K, Vieira J, Moreira A, et al. Stereotypies in Rett syndrome: analysis of 83 patients with and without detected MECP2 mutations. Neurology. 2007;68:1183–7.CrossRefPubMed

31.

National Institute of Neurological Disorders and Stroke. Rett syndrome fact sheet. 2009. https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Rett-Syndrome-Fact-Sheet. Accessed 18 May 2017.

32.

Tonetti L, Pasquini F, Fabbri M, Belluzzi M, Natale V. Comparison of two different actigraphs with polysomnography in healthy young subjects. Chronobiol Int. 2008;25:145–53. https://doi.org/10.1080/07420520801897228.CrossRefPubMed

33.

Fritz CO, Morris PE, Richler JJ. Effect size estimates: current use, calculations, and interpretation. J Exp Psychol Gen. 2012;141:2–18. https://doi.org/10.1037/a0024338.CrossRefPubMed

34.

Paruthi S, Brooks LJ, D’Ambrosio C, Hall WA, Kotagal S, Lloyd RM, et al. Consensus statement of the American Academy of Sleep Medicine on the recommended amount of sleep for healthy children: methodology and discussion. J Clin Sleep Med. 2016;12:1549–61.CrossRefPubMedPubMedCentral

35.

Hirshkowitz M, Whiton K, Albert SM, Alessi C, Bruni O, DonCarlos L, et al. National Sleep Foundation’s updated sleep duration recommendations: final report. Sleep Heal. 2015;1:233–43. https://doi.org/10.1016/j.sleh.2015.10.004.CrossRef

36.

Crabtree VM, Williams NA. Normal sleep in children and adolescents. Child Adolesc Psychiatr Clin N Am. 2009;18:799–811.CrossRef

37.

Noor ZM, Smith AJ, Smith SS, Nissen LM. Feasibility and acceptability of wrist actigraph in assessing sleep quality and sleep quantity: a home-based pilot study in healthy volunteers. Health. 2013;5 8A2:63–72.

38.

Barney CC, Feyma T, Beisang A, Symons FJ. Pain experience and expression in Rett syndrome: subjective and objective measurement approaches. J Dev Phys Disabil. 2015;27:417–29.CrossRefPubMedPubMedCentral

39.

Symons FJ, Byiers B, Tervo RC, Beisang A. Parent-reported pain in Rett syndrome. Clin J Pain. 2013;29:744–6. https://doi.org/10.1097/AJP.0b013e318274b6bd.CrossRefPubMedPubMedCentral

40.

Surtees A, Oliver C, Jones C, Psyd C, Evans D, Richards C. Sleep duration and sleep quality in people with and without intellectual disability: a meta-analysis. Sleep Med Rev. 2017; https://doi.org/10.1016/j.smrv.2017.11.003.

41.

Didden R, Sigafoos J. A review of the nature and treatment of sleep disorders in individuals with developmental disabilities. Res Dev Disabil. 2001;22:255–72.CrossRefPubMed

42.

Malow BA. The interaction between sleep and epilepsy. Epilepsia. 2007;48(SUPPL. 9):36–8.CrossRefPubMed

43.

Newman CJ, O’Regan M, Hensey O. Sleep disorders in children with cerebral palsy. Dev Med Child Neurol. 2018;48:564–8.CrossRef

44.

Simard-Tremblay E, Constantin E, Gruber R, Brouillette RT, Shevell M. Sleep in children with cerebral palsy: a review. J Child Neurol. 2011;26:1303–10.CrossRefPubMed

45.

Hoffman CD, Sweeney DP, Gilliam JE, Apodaca DD, Lopez-Wagner MC, Castillo MM. Sleep problems and symptomology in children with autism. Focus Autism Other Deveolpmental Disabil. 2005;20:194–200.CrossRef

46.

Ohayon MM, Carskadon MA, Guilleminault C, Vitiello MV. Meta-analysis of quantitative sleep parameters from childhood to old age in healthy individuals: developing normative sleep values across the human lifespan. Sleep. 2004;27:1255–73.

47.

Carotenuto M, Esposito M, D’Aniello A, Rippa CD, Precenzano F, Pascotto A, et al. Polysomnographic findings in Rett syndrome: a case-control study. Sleep Breath. 2013;17:93–8.

48.

Sadeh A. Commentary: comparing actigraphy and parental report as measures of children’s sleep. J Pediatr Psychol. 2008;33:406–7.CrossRefPubMed

Title: The feasibility of using actigraphy to characterize sleep in Rett syndrome
Authors: Alyssa M. Merbler
Breanne J. Byiers
John J. Garcia
Timothy J. Feyma
Frank J. Symons
Publication date: 01-12-2018
Publisher: BioMed Central
Published in: Journal of Neurodevelopmental Disorders / Issue 1/2018
Print ISSN: 1866-1947
Electronic ISSN: 1866-1955
DOI: https://doi.org/10.1186/s11689-018-9227-z

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

The feasibility of using actigraphy to characterize sleep in Rett syndrome

Abstract

Background

Methods

Results

Conclusions

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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