Novel insights into maladaptive behaviours in Prader-Willi syndrome: serendipitous findings from an open trial of vagus nerve stimulation.
McAllister, Catherine J
Kelly, Claire L
Sylvester, Karl P
Garnett, Matthew R
Manford, Mark RA
Journal of Intellectual Disability Research
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Manning, K., McAllister, C. J., Ring, H., Finer, N., Kelly, C. L., Sylvester, K. P., Fletcher, P., et al. (2015). Novel insights into maladaptive behaviours in Prader-Willi syndrome: serendipitous findings from an open trial of vagus nerve stimulation.. Journal of Intellectual Disability Research, 60 149-155. https://doi.org/10.1111/jir.12203
Background: We report striking and unanticipated improvements in maladaptive behaviours in Prader–Willi syndrome (PWS) during a trial of vagus nerve stimulation (VNS) initially designed to investigate effects on the overeating behaviour. PWS is a genetically determined neurodevelopmental disorder associated with mild–moderate intellectual disability (ID) and social and behavioural difficulties, alongside a characteristic and severe hyperphagia. Methods: Three individuals with PWS underwent surgery to implant the VNS device. VNS was switched on 3 months post-implantation, with an initial 0.25 mA output current incrementally increased to a maximum of 1.5 mA as tolerated by each individual. Participants were followed up monthly. Results: Vagal nerve stimulation in these individuals with PWS, within the stimulation parameters used here, was safe and acceptable. However, changes in eating behaviour were equivocal. Intriguingly, unanticipated, although consistent, beneficial effects were reported by two participants and their carers in maladaptive behaviour, temperament and social functioning. These improvements and associated effects on food-seeking behaviour, but not weight, indicate that VNS may have potential as a novel treatment for such behaviours. Conclusions: We propose that these changes are mediated through afferent and efferent vagal projections and their effects on specific neural networks and functioning of the autonomic nervous system and provide new insights into the mechanisms that underpin what are serious and common problems affecting people with IDs more generally.
Prader-Willi syndrome, vagus nerve stimulation, hyperphagia, maladaptive behaviour, social functioning
This study was funded by The Dunhill Medical Trust, Addenbrooke’s Charitable Trust, Isaac Newton Trust , and Prader-Willi Association UK. Funding bodies had no role in study design, data collection, data analysis, data interpretation, writing of the report or the decision to submit for publication. We are grateful to the NIHR Collaborations for Leadership in Applied Health Care Research and Care (CLAHRC) East of England for financial support to AJH and HAR and to the Health Foundation for support of AJH. The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health.
Medical Research Council (MC_UU_12012/5/B)
Health Foundation (543/1163)
Foundation for Prader-Willi Research (unknown)
Wellcome Trust (095692/Z/11/Z)
External DOI: https://doi.org/10.1111/jir.12203
This record's URL: https://www.repository.cam.ac.uk/handle/1810/248254
Attribution 2.0 UK: England & Wales
Licence URL: http://creativecommons.org/licenses/by/2.0/uk/