Rett Disorder and the Developing Brain

Paperback | April 28, 2005

EditorAlison Kerr, Ingegerd Witt Engerstrom

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Rett disorder is a devastating neurodevelopmental disorder that most exclusively affects girls, and is the second most common cause of severe intellectual disability in females after Down syndrome. Its many manifestations include epilepsy, scoliosis, nutritional difficulties, and disorders ofmood and development. This new paperback edition of Rett Disorder and the Developing Brain provides an up-to-date account of current research into the disorder. The first section presents clinical signs and the latest research into the genetic, neuro-anatomical and neurochemical aspects of Rett disorder. The second section moves on to deal with the neurophysiological research, concentrating on the autonomic system, which is deeply implicated in the diseasemechanisms. The final section examines the behavioural and psychological features and discusses issues relating to communication. The chapters are written by a distinguished, international team of experts, all currently active in research. The contributions bring our understanding of Rett disorderright up-to-date, as the study of the condition moves from an era of description into a time when there are real possibilities for beneficial interventions.

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From the Publisher

Rett disorder is a devastating neurodevelopmental disorder that most exclusively affects girls, and is the second most common cause of severe intellectual disability in females after Down syndrome. Its many manifestations include epilepsy, scoliosis, nutritional difficulties, and disorders ofmood and development. This new paperback edi...

Alison Kerr is at the Academic Centre, Department of Psychological Medicine, Gartnaval Royal Hospital, Glasgow, UK. Ingegerd Witt Engerstrom is a Senior Consultant in Paediatric Neurology and Habilitation, The Rett Centre, Froson, Sweden.
Format:PaperbackDimensions:398 pages, 9.45 × 6.61 × 0.91 inPublished:April 28, 2005Publisher:Oxford University PressLanguage:English

The following ISBNs are associated with this title:

ISBN - 10:0198568150

ISBN - 13:9780198568155

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Table of Contents

1. Alison Kerr and Ingegerd Witt Engerstrom: The clinical background to the Rett disorderRoots of Disorder2. Angus Clarke, Carolyn Schanen and Maria Anvret: Towards the genetic basis of Rett syndrome3. Dawna D. Armstrong and Hannah C. Kinney: The neuropathology of the Rett disorder4. Walter E. Kaufmann: Cortical development in Rett syndrome: molecular, neurochemical, and anatomical aspects5.1. Maj A. Hulten: The Rett syndrome: proposed mechanisms of genetic origin and inheritance5.2. Mary E. Blue and Michael V. Johnston: Amino acid receptor studies in Rett syndrome5.3. Sarojini S. Budden: Melatonin and the Rett syndrome disorder5.4. Souad Messahel, Anne E. Pheasant, Hardiv Pall and Alison Kerr: Early abnormality in pterin levels in Rett syndrome5.5. Raili Riikonen: Neurotrophic factors in the pathogenesis of Rett syndromeNeurones in Action6. Peter O. O. Julu: The central autonomic disturbance in Rett syndrome7. Masaya Segawa: The role of genetic and environmental factors in brain development: development of the central monaminergic nervous system8. Yoshiko Nomura and Masaya Segawa: The monamine hypothesis in Rett syndrome9. Annica Dahlstrom: The central and peripheral autonomic nervous system and possible implications in Rett syndrome patients10.1. Daniel G. Glaze and Rebecca J. Schultz: Autonomic dysfunction and sudden death in Rett syndrome: prolonged QTc intervals and diminished heart rate variability10.2. Richard Morton: Feeding in Rett syndrome10.3. Kathleen J. Motil, Rebecca J. Schultz, Daniel G. Glaze and Dawna Armstrong: Oropharyngeal dysfunction and upper gastrointestinal dysmotility, a reflection of disturbances in the autonomic nervous system in Rett syndrome10.4. Helen Leonard, Susan Fyfe and Carolyn Ellaway: Possible link between skeletal and electrocardiographic abnormalities and autonomic dysfunction in Rett syndrome10.5. Rosemary A. Cooper: The electroencephalogram in Rett syndrome10.6. Urban M. Fietzek, Florian Heinen, U. Ziemann, H. Petersen, K. Huhn, J. Schulte-Monting, H-J. Christen, R. Korinthenberg and F. Hanefeld: Electromagnetic stimulation of motor neuronsExpressing Intelligence11. Pavel V. Belichenko: The morphological substrate for communication12. Colwyn Trevarthen and Bronwen Burford: Early infant intelligence and Rett syndrome13. Bjorn Merker and Nils L. Wallin: Musical responsiveness in the Rett disorder14.1. Rebecca Mount, Richard Hastings, Tony Charman, Sheena Reilly and Hilary Cass: Behavioural and emotional features of Rett syndrome14.2. Daphne L. McCulloch, Ross M. Henderson, Kathryn J. Saunders and R. M. Walley: Vision in Rett syndrome: studies using evoked potentials and event-related potentialsConcluding chapter15. Alison M. Kerr and Ingegerd Witt Engerstrom: The developmental perspective in Rett disorder: where next?

Editorial Reviews

`Kerr and Engerstrom succeed in providing a comprehensive account of current knowledge... This book will be a valuable guide for clinicians and researchers of human disease in various specialties. Clinicians, educators, therapists and families affected by this enigmatic disorder... willbenefit from the knowledge this book contributes to advice and management.'The Lancet