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 of mood 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 disease mechanisms. The final section examines the behavioural and psychological features and discusses issues relating to communication. The chapters are written by a distinguished, international team ofexperts, all currently active in research. The contributions bring our understanding of Rett disorder right 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.
The clinical background to the Rett disorder; Roots of Disorder; Towards the genetic basis of Rett syndrome; The neuropathology of the Rett disorder; Cortical development in Rett syndrome: molecular, neurochemical, and anatomical aspects; The Rett syndrome: proposed mechanisms of genetic origin and inheritance; Amino acid receptor studies in Rett syndrome; Melatonin and the Rett syndrome disorder; Early abnormality in pterin levels in Rett syndrome; Neurotrophic factors in the pathogenesis of Rett syndrome; Neurones in Action; The central autonomic disturbance in Rett syndrome; The role of genetic and environmental factors in brain development: development of the central monaminergic nervous system; The monamine hypothesis in Rett syndrome; The central and peripheral autonomic nervous system and possible implications in Rett syndrome patients; Autonomic dysfunction and sudden death in Rett syndrome: prolonged QTc intervals and diminished heart rate variability; Feeding in Rett syndrome; Oropharyngeal dysfunction and upper gastrointestinal dysmotility, a reflection of disturbances in the autonomic nervous system in Rett syndrome; Possible link between skeletal and electrocardiographic abnormalities and autonomic dysfunction in Rett syndrome; The electroencephalogram in Rett syndrome; Electromagnetic stimulation of motor neurons; Expressing Intelligence; The morphological substrate for communication; Early infant intelligence and Rett syndrome; Musical responsiveness in the Rett disorder; Behavioural and emotional features of Rett syndrome; Vision in Rett syndrome: studies using evoked potentials and event-related potentials; Concluding chapter; The developmental perspective in Rett disorder: where next?;
Comments (0)
Your review appreciation cannot be sent
Report comment
Are you sure that you want to report this comment?
Report sent
Your report has been submitted and will be considered by a moderator.