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Puzzle Pieces: Neural Structure and Function in Prader-Willi Syndrome

Department of Psychiatry, University of Cambridge, Cambridge, CB2 8AH, UK
National Institute for Health Research (NIHR) Collaborations for Leadership in Applied Health Research and Care (CLAHRC) East of England, Cambridge, CB2 8AH, UK
Cambridgeshire and Peterborough Mental Health Services (NHS) Foundation Trust, Cambridge, CB21 5EF, UK
Author to whom correspondence should be addressed.
Academic Editor: Danny Camfferman
Diseases 2015, 3(4), 382-415;
Received: 1 October 2015 / Revised: 4 December 2015 / Accepted: 11 December 2015 / Published: 17 December 2015
(This article belongs to the Special Issue Prader-Willi Syndrome)
Prader-Willi syndrome (PWS) is a neurodevelopmental disorder of genomic imprinting, presenting with a behavioural phenotype encompassing hyperphagia, intellectual disability, social and behavioural difficulties, and propensity to psychiatric illness. Research has tended to focus on the cognitive and behavioural investigation of these features, and, with the exception of eating behaviour, the neural physiology is currently less well understood. A systematic review was undertaken to explore findings relating to neural structure and function in PWS, using search terms designed to encompass all published articles concerning both in vivo and post-mortem studies of neural structure and function in PWS. This supported the general paucity of research in this area, with many articles reporting case studies and qualitative descriptions or focusing solely on the overeating behaviour, although a number of systematic investigations were also identified. Research to date implicates a combination of subcortical and higher order structures in PWS, including those involved in processing reward, motivation, affect and higher order cognitive functions, with both anatomical and functional investigations indicating abnormalities. It appears likely that PWS involves aberrant activity across distributed neural networks. The characterisation of neural structure and function warrants both replication and further systematic study. View Full-Text
Keywords: Prader-Willi syndrome; brain; systematic review; neuroimaging; post-mortem Prader-Willi syndrome; brain; systematic review; neuroimaging; post-mortem
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Manning, K.E.; Holland, A.J. Puzzle Pieces: Neural Structure and Function in Prader-Willi Syndrome. Diseases 2015, 3, 382-415.

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