Daytime sleepiness and emotional and behavioral disturbances in Prader-Willi syndrome

Catherine S Choong; Gillian M Nixon; A Marie Blackmore; Wai Chen; Peter Jacoby; Helen Leonard; Antony R Lafferty; Geoff Ambler; Nitin Kapur; Philip B Bergman; Cara Schofield; Chris Seton; Andrew Tai; Elaine Tham; Komal Vora; Patricia Crock; Charles Verge; Yassmin Musthaffa; Greg Blecher; Andrew Wilson; Jenny Downs

doi:10.1007/s00431-022-04439-2

Daytime sleepiness and emotional and behavioral disturbances in Prader-Willi syndrome

Eur J Pediatr. 2022 Jun;181(6):2491-2500. doi: 10.1007/s00431-022-04439-2. Epub 2022 Mar 22.

Authors

Catherine S Choong^{1

2

3}, Gillian M Nixon^{4

5

6}, A Marie Blackmore^{1

7}, Wai Chen^{8

9}, Peter Jacoby¹, Helen Leonard¹, Antony R Lafferty^{10

11}, Geoff Ambler^{12

13}, Nitin Kapur^{14

15}, Philip B Bergman^{5

6}, Cara Schofield^{1

16}, Chris Seton^{17

18}, Andrew Tai¹⁹, Elaine Tham¹⁹, Komal Vora^{20

21}, Patricia Crock^{20

21

22}, Charles Verge^{12

23}, Yassmin Musthaffa^{15

14

24}, Greg Blecher²⁵, Andrew Wilson^{1

2

3}, Jenny Downs^{26

27

28}

Affiliations

¹ Telethon Kids Institute, 15 Hospital Avenue, Nedlands, Western Australia, 6009, Australia.
² Perth Children's Hospital, Nedlands, Western Australia, Australia.
³ The University of Western Australia, Nedlands, Western Australia, Australia.
⁴ Sophia Children's Hospital, Erasmus University Medical Centre, Rotterdam, Netherlands.
⁵ Monash Children's Hospital, Clayton, Victoria, Australia.
⁶ Monash University, Clayton, Victoria, Australia.
⁷ Child and Adolescent Health Service, Perth, Western Australia, Australia.
⁸ Fiona Stanley Hospital, Murdoch, Western Australia, Australia.
⁹ Curtin University, Bentley, Western Australia, Australia.
¹⁰ Canberra Hospital, Garran, Capital Territory, Australia.
¹¹ ANU Medical School, Canberra, Capital Territory, Australia.
¹² The Sydney Children's Hospitals Network, Westmead, New South Wales, Australia.
¹³ The University of Sydney, Sydney CBD, New South Wales, Australia.
¹⁴ Queensland Children's Hospital, South Brisbane, Queensland, Australia.
¹⁵ The University of Queensland, Brisbane, Queensland, Australia.
¹⁶ La Trobe University, Bundoora, Victoria, Australia.
¹⁷ The Children's Hospital at Westmead, Westmead, New South Wales, Australia.
¹⁸ Woolcock Institute of Medical Research, Glebe, New South Wales, Australia.
¹⁹ Women's and Children's Hospital, North Adelaide, South Australia, Australia.
²⁰ John Hunter Children's Hospital, Newcastle, New South Wales, Australia.
²¹ The University of Newcastle, Newcastle, New South Wales, Australia.
²² Hunter Medical Research Institute, Newcastle, NSW, Australia.
²³ The University of New South Wales, Kennington, New South Wales, Australia.
²⁴ Logan Hospital, Meadowbrook, Queensland, Australia.
²⁵ The Sydney Children's Hospitals Network, Randwick, New South Wales, Australia.
²⁶ Telethon Kids Institute, 15 Hospital Avenue, Nedlands, Western Australia, 6009, Australia. Jenny.Downs@telethonkids.org.au.
²⁷ Curtin University, Bentley, Western Australia, Australia. Jenny.Downs@telethonkids.org.au.
²⁸ The University of Western Australia, Nedlands, Western Australia, Australia. Jenny.Downs@telethonkids.org.au.

Abstract

Individuals with Prader-Willi syndrome (PWS) often have excessive daytime sleepiness and emotional/behavioral disturbances. The objective of this study was to examine whether daytime sleepiness was associated with these emotional/behavioral problems, independent of nighttime sleep-disordered breathing, or the duration of sleep. Caregivers of individuals with PWS (aged 3 to 25 years) completed the Pediatric Sleep Questionnaire (PSQ), Epworth Sleepiness Scale for Children and Adolescents (ESS-CHAD), and the parent version of the Developmental Behavior Checklist (DBC-P). Sleep adequacy was adjusted for age by computing sleep duration against age-specific recommendations. The associations between ESS-CHAD and the total DBC and its subscale scores were evaluated by linear regression, adjusted for sleep-related breathing difficulties, sleep adequacy, and body mass index (BMI). There were 54 responses for individuals with PWS (including 22 males) aged 4.4-24.0 (mean 12.5) years. Daytime sleepiness predicted a substantial proportion of the variance in total DBC-P scores in the unadjusted model (28%; β = 0.028; p < 0.001) and when adjusted for sleep adequacy, BMI, and sleep-related breathing difficulties (29%; β = 0.023; p = 0.007). This relationship was not moderated by BMI Z-scores, but the relationship was more prominent for children younger than 12 years than for children older than 12 years.Conclusions: These findings provide preliminary novel evidence that daytime sleepiness may drive the expression of emotional/behavioral disturbances, and should be explored as a potential modifiable risk factor for these disturbances in PWS, particularly pre-adolescent children.

Keywords: Genetic disorder; Intellectual disability; Mental health; Sleepiness.

MeSH terms

Adolescent
Child
Disorders of Excessive Somnolence* / complications
Emotions
Humans
Male
Prader-Willi Syndrome* / complications
Problem Behavior*
Sleep