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A connective tissue disorder may underlie ESSENCE problems in childhood

Carolina Baeza-Velasco1, Rodney Grahame2, Jaime F Bravo3

  • 1Department of Psychology, Laboratory of Psychopathology and Health Process, University Paris Descartes - Sorbonne Paris Cité, Boulogne-Billancourt, France.

Research in Developmental Disabilities
|November 3, 2016
PubMed

Insights

Children with Ehlers-Danlos syndrome hypermobility type (EDS-HT/JHS) often experience neurodevelopmental challenges, known as ESSENCE problems. These issues may be recognized before the underlying connective tissue disorder is diagnosed, highlighting the need for increased awareness.

Area of Science:

  • Pediatric Rheumatology
  • Neurodevelopmental Pediatrics
  • Genetics

Background:

  • Ehlers-Danlos syndrome hypermobility type (EDS-HT/JHS) is a common hereditary connective tissue disorder (HDCT) with varied symptoms.
  • EDS-HT/JHS involves tissue fragility, joint hypermobility, and numerous other manifestations, often starting in infancy.
  • The medical community has limited awareness of EDS-HT/JHS and the associated Early Symptomatic Syndromes Eliciting Neurodevelopmental Clinical Examinations (ESSENCE) problems in children.

Purpose of the Study:

  • To review the clinical and empirical evidence linking EDS-HT/JHS in children to ESSENCE difficulties.
  • To synthesize current knowledge on the co-occurrence of neurodevelopmental issues and EDS-HT/JHS.

Main Methods:

  • A narrative literature review was conducted.
  • Searches encompassed scientific online databases and reference lists, including quantitative and qualitative research.

Main Results:

  • Evidence suggests an association between EDS-HT/JHS and ESSENCE difficulties.
  • These include motor abnormalities, hyperactivity/hypoactivity, inattention, speech/language issues, social interaction challenges, behavioral problems, sleep disturbances, feeding difficulties, and emotional problems.

Conclusions:

  • Children with EDS-HT/JHS frequently present with ESSENCE problems, which are often identified before the HDCT diagnosis.
  • Clinicians should consider EDS-HT/JHS in children with ESSENCE problems, as it may impact neurodevelopment.
  • Recognizing these interconnected issues can improve early diagnosis and treatment of EDS-HT/JHS.
Abstract

Keywords:
A typical brain developmentAttention deficitDevelopmental coordination disorderESSENCEEhlers-Danlos syndromeFeedingHyperactivityHypoactivityJoint hypermobility syndromeNeurodevelopmental disordersSleep problems

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