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Sleep and behavior in children and adolescents with tuberous sclerosis complex

Elena Zambrelli1, Katherine Turner1, Angela Peron1,2,3,4

  • 1Epilepsy Center-Sleep Medicine Center, Childhood and Adolescence Neuropsychiatry Unit, ASST SS. Paolo e Carlo, San Paolo Hospital, Milan, Italy.

American Journal of Medical Genetics. Part A
|March 2, 2021
PubMed

Insights

Sleep disorders are common in children with tuberous sclerosis complex (TSC), affecting nearly half of patients. These sleep disturbances are linked to behavioral issues, suggesting integrated monitoring is crucial for TSC care.

Area of Science:

  • Pediatric Neurology
  • Sleep Medicine
  • Genetics

Background:

  • Sleep disorders are prevalent in children with tuberous sclerosis complex (TSC) but lack detailed characterization.
  • Tuberous sclerosis complex is a genetic disorder affecting multiple organs, including the brain, often leading to neurological and developmental challenges.

Purpose of the Study:

  • To investigate the prevalence and characteristics of sleep disorders in children with TSC.
  • To explore the relationship between sleep disturbances and behavioral problems in this population.

Main Methods:

  • A cohort of 46 children with TSC and 46 age- and sex-matched healthy controls participated.
  • Parents completed the Sleep Disturbances Scale for Children (SDSC) and the Child Behavior Checklist (CBCL).
  • Statistical analyses were performed to compare sleep and behavioral scores between groups and assess correlations.

Main Results:

  • 17.4% of TSC patients had abnormal total scores on the SDSC, compared to 4.4% in controls (p=0.024).
  • 45.7% of TSC patients reported at least one abnormal factor score on the SDSC.
  • Significant differences were observed in most CBCL scale scores between TSC patients and controls.
  • Total SDSC scores strongly correlated with Total CBCL scores (R-square=0.387) and its Internalizing/Externalizing areas (R-square=0.291, R-square=0.350).

Conclusions:

  • Sleep disorders are significantly more frequent in children with TSC than in the general population.
  • Sleep disturbances in TSC are closely associated with behavioral problems.
  • The SDSC and CBCL are recommended as part of routine surveillance for children with TSC.

Keywords:
CBCLSDSCTSCsleeptuberous sclerosis complex

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