title: Reflections on the constraints and opportunities in therapy in Rett syndrome.
abstract: More than 20 years of clinical and research experience with affected people in the British Isles has provided insight into particular challenges for therapists, educators, or parents wishing to facilitate learning and to support the development of skills in people with Rett syndrome. This paper considers the challenges in two groups: those due to constraints imposed by the disabilities associated with the disorder and those stemming from the opportunities, often masked by the disorder, allowing the development of skills that depend on less-affected areas of the brain. Because the disorder interferes with the synaptic links between neurones, the functions of the brain that are most dependent on complex neural networks are the most profoundly affected. These functions include speech, memory, learning, generation of ideas, and the planning of fine movements, especially those of the hands. In contrast, spontaneous emotional and hormonal responses appear relatively intact. Whereas failure to appreciate the physical limitations of the disease leads to frustration for therapist and client alike, a clear understanding of the better-preserved areas of competence offers avenues for real progress in learning, the building of satisfying relationships, and achievement of a quality of life.
authors.affiliation: Section of Psychological Medicine, Division of Community Based Sciences, Academic Centre, Gartnavel Royal Hospital, 1055 Great Western Road, Glasgow G12 0XH, UK. amk5m@clinmed.gla.ac.uk
authors.forename: Alison M
authors.initials: AM
authors.lastname: Kerr
mesh_terms: D000339:Affect; D002648:Child; D003142:Communication; D005260:Female; D006321:Heart / Q000502:physiology; D006801:Humans; D008297:Male; D009068:Movement; D009747:Nutritional Physiological Phenomena; D011788:Quality of Life; D012119:Respiration; D015518:Rett Syndrome / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy*