A systematic review of non-medical interventions in Rett Syndrome and a research study into attenuated behaviours in Rett Syndrome

• Main Author: Amoako, Annika Nina
• Published: Cardiff University 2018
• Subjects: BF Psychology
• Online Access: https://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.761319

This thesis was submitted in May 2018 for the partial fulfilment of the award of Doctor in Clinical Psychology (DClinPsy) at Cardiff University. The thesis investigated a review of non-medical interventions in Rett Syndrome and the prevalence of attenuated behaviours in Rett Syndrome. Rett Syndrome is a rare neurodevelopmental genetic disorder which is characterised by period of outwardly typical development followed by a period of regression around 12-18 months. The regression is causes progressive disabilities in speech, motor and hand use. Physical comorbidities are often present including breathing difficulties, the requirement of feeding tubes, anxiety, gastrointestinal difficulties and orthopaedic issues. The condition is almost exclusive to females. Despite the inability to use their body to communicate, research has shown that individuals with Rett Syndrome are more intellectually capable than their body allows them to present. Paper 1 describes a systematic review of non-medical interventions researched into Rett Syndrome. The electronic databases were searched (Embase, PsychINFO and MEDLINE). Thirteen papers which met the quality rating threshold were reviewed and the methodology was assessed. Communication interventions were the most researched intervention. Alternative interventions included fitness and brainstem activation. Eleven of the studies described positive results. Recommendations for clinical practice and future research are made. Paper 2 describes an empirical study investigating the prevalence of attenuated behaviours [Autistic Catatonia] in Rett Syndrome and its presence during ‘Rett Episodes’. Parents of 28 individuals with Rett Syndrome completed questionnaires relating to attenuated behaviours and Rett Episodes. The findings revealed the presence of attenuated behaviour in individuals with Rett Episodes but this was not specific to Rett Episodes. The severity of attenuated behaviour was negatively correlated with age. Recommendations for clinical implications and further research are made. Paper 3 discusses an evaluation of the research process. The paper critically appraises the research across both papers including the strengths and limitations. The paper includes key reflections and process information that was not permitted within the constraints of the author guidelines for submission.