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Related Experiment Videos

Self-injurious behavior.

Sylvia Huisman1, Paul Mulder2, Janneke Kuijk3

  • 1Department of Pediatrics, Academic Medical Center, University of Amsterdam, Meibergdreef 9, 1105 AZ Amsterdam, the Netherlands; Prinsenstichting, Kwadijkerpark 8, 1444 JE Purmerend, the Netherlands.

Neuroscience and Biobehavioral Reviews
|July 12, 2017
PubMed
Summary

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Self-injurious behavior (SIB) is common in intellectual disabilities (ID). This review highlights higher SIB prevalence and varied characteristics in genetic syndromes, suggesting distinct underlying pathways for targeted interventions.

Area of Science:

  • Neuroscience
  • Genetics
  • Behavioral Science

Background:

  • Self-injurious behavior (SIB) is prevalent in individuals with intellectual disabilities (ID).
  • Severe SIB poses significant health risks.
  • Emerging evidence links pain and discomfort to SIB in genetic syndromes.

Purpose of the Study:

  • To review SIB phenomenology in individuals with ID and twelve genetic syndromes.
  • To summarize SIB characteristics and influencing factors across distinct genetic entities.
  • To identify genetic pathways contributing to SIB for intervention development.

Main Methods:

  • Literature review of SIB in ID and specific genetic syndromes.
  • Comparative analysis of SIB prevalence, onset, and topographies.
Keywords:
Angelman syndromeCornelia de Lange SyndromeCri du Chat SyndromeDown syndromeFragile X syndromeGenesGenetic syndromesIntellectual disabilityInterdisciplinary studyLesch-Nyhan syndromeLowe syndromePainPrader-Willi syndromeRett syndromeSelf-injurious behaviorSmith-Magenis syndromeSomatic factorsTuberous Sclerosis SyndromeWilliams-Beuren syndrome

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  • Examination of genetic underpinnings and associated biological pathways.
  • Main Results:

    • SIB prevalence is notably higher in several genetic ID syndromes compared to general ID.
    • SIB characteristics, including age of onset and topographies, vary significantly across syndromes.
    • Each genetic syndrome involves distinct gene mutations, suggesting diverse etiological pathways.

    Conclusions:

    • Genetic syndromes present unique SIB profiles.
    • Understanding syndrome-specific pathways is crucial for developing targeted early interventions and prevention strategies for SIB.