Psychiatric and psychological aspects in the Ehlers-Danlos syndromes

Am J Med Genet C Semin Med Genet. 2017 Mar;175(1):237-245. doi: 10.1002/ajmg.c.31544. Epub 2017 Feb 10.


There is increasing amount of evidence pointing toward a high prevalence of psychiatric conditions among individuals with hypermobile type of Ehlers-Danlos syndrome (JHS/hEDS). A literature review confirms a strong association between anxiety disorders and JHSh/hEDS, and there is also limited but growing evidence that JHSh/hEDS is also associated with depression, eating, and neuro-developmental disorders as well as alcohol and tobacco misuse. The underlying mechanisms behind this association include genetic risks, autonomic nervous system dysfunction, increased exteroceptive and interoceptive mechanisms and decreased proprioception. Recent neuroimaging studies have also shown an increase response in emotion processing brain areas which could explain the high affective reactivity seen in JHS/hEDS. Management of these patients should include psychiatric and psychological approaches, not only to relieve the clinical conditions but also to improve abilities to cope through proper drug treatment, psychotherapy, and psychological rehabilitation adequately coupled with modern physiotherapy. A multidimensional approach to this "neuroconnective phenotype" should be implemented to ensure proper assessment and to guide for more specific treatments. Future lines of research should further explore the full dimension of the psychopathology associated with JHS/hEDS to define the nature of the relationship. © 2017 Wiley Periodicals, Inc.

Keywords: anxiety; hypermobile Ehlers-Danlos syndrome; joint hypermobility; neuroconnective phenotype; psychopathology.

Publication types

  • Review

MeSH terms

  • Anxiety Disorders / therapy
  • Behavioral Symptoms / therapy
  • Ehlers-Danlos Syndrome / psychology*
  • Humans
  • Mental Disorders / therapy
  • Practice Guidelines as Topic