REVIEW article
Front. Neurol.
Sec. Autonomic Disorders
Volume 16 - 2025 | doi: 10.3389/fneur.2025.1648702
Neurobiological and neuropsychological disturbance in EDS
Provisionally accepted
- 1School of Health Sciences, Faculty of Biology, Medicine and Health, The Andrew Mayes Centre for Cogntion and Neuroscience, The University of Manchester, Manchester, United Kingdom
- 2Geoffrey Jefferson Brain Research Centre, Manchester Academic Health Science Centre, Northern Care Alliance & University of Manchester, Manchester, UK; Division of Cardiovascular Sciences, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, M13 9PL, UK, The University of Manchester, Manchester, United Kingdom
- 3College of Health and Life Sciences, Aston University, Birmingham, United Kingdom
- 4Centre for Cognition and Neuroscience, Department of Psychology, The University of Huddersfield, Huddersfield, UK, University of Huddersfield, Huddersfield, United Kingdom
- 5School of Education, Language and Psychology, York St John University, York, United Kingdom
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Ehlers-Danlos Syndrome (EDS) is a collection of connective tissue disorders, defined by genetic defects in collagen and extracellular matrix proteins that lead to joint hypermobility, skin fragility, and vascular complications. However, recent studies point to a broader impact, revealing how EDS has both neurological and psychological effects. This review explores these neurological and neuropsychological dimensions of EDS across its 13 subtypes, drawing together evidence on brain structure changes such as Chiari malformations and craniocervical instability, alongside small fibre neuropathy, blood-brain barrier vulnerabilities, and cerebrovascular risks, particularly prevalent in the vascular EDS subtype. The review will also explore how these physical disruptions may act upon mental health, fueling anxiety, mood instability, and cognitive challenges. Mechanisms such as neuroinflammation, altered interoception, and chronic pain may contribute to these effects and drive emotional dysregulation. By reviewing clinical observations, neuroimaging findings, and emerging theories, this paper highlights the importance of understanding the involvement of the brain in EDS. The review highlights the need for a shift in approach to EDS, and an integrated effort across neurology, psychiatry, and genetics to better support those living EDS.
Keywords: Ehlers Danlos syndrome, emotional dysregulation, Anxiety, Chronic Pain, Chairi malformation, cerebrovascular, interoception
Received: 16 Jul 2025; Accepted: 16 Oct 2025.
Copyright: © 2025 Westerman, Kafkas, Parry-Jones, Strong, Retzler and Hallam. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Matt Westerman, matt.westerman@manchester.ac.uk
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