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Neurophysiological responses to music and vibroacoustic stimuli in Rett syndrome
Institution:1. Rett Center Jämtland County Council, Östersund, Sweden;2. Department of Women and Child Health, Umea University, Sweden;3. Department of Communication and Psychology, Aalborg University, Denmark;4. GAMUT, Uni Health, Uni Research, Bergen, Norway;1. Department of Clinical Child and Family Studies and LEARN! Research Institute for Learning and Education, VU University Amsterdam, The Netherlands;3. Tranzo Scientific Center for Care and Welfare and Department of Medical and Clinical Psychology, Tilburg University, The Netherlands;4. De Bascule, Academic Center for Child and Adolescent Psychiatry, Amsterdam, The Netherlands;5. Department of Child and Adolescent Studies, Utrecht University, and Department of Psychiatry, University Medical Center Utrecht, The Netherlands;1. Department of Orthopaedic and Trauma Surgery, Heidelberg University Clinics, Heidelberg, Germany;2. Department of Trauma Surgery and Orthopaedics, University Medical Center Goettingen, Goettingen, Germany;1. The Nisonger Center UCEDD, Ohio State University, McCampbell Hall, 1581 Dodd Dr., Columbus, OH 43210, United States;2. Division of Neurodevelopmental and Behavioral Pediatrics at the University of Rochester, Box 671, 601 Elmwood Avenue, Rochester, NY 14642, United States;3. Department of Psychiatry at the Western Psychiatric Institute and Clinic, Thomas Detre Hall, 3811 O‘Hara Street, Pittsburgh, PA 15213, United States;4. Center for Biostatistics, Ohio State University, 2012 Kenny Road, Columbus, OH 43221, United States;1. University at Buffalo, State University of New York, Buffalo, NY 14260, USA;2. St. Mary׳s College of Maryland, St. Mary׳s City, MD, USA;3. McMaster University, Hamilton, ON, Canada;4. Buffalo Neuroimaging Analysis Center, Buffalo, NY, USA;1. Department of Occupational Therapy, Faculty of Social Welfare and Health Sciences, University of Haifa, Haifa 31905, Israel;2. Pediatric Neurology Unit and the Gilbert Israeli Neurofibromatosis Center (GINFC), Dana Children''s Hospital, Tel Aviv Sourasky Medical Center, 6 Weizmann Street, Tel Aviv 64239, Israel
Abstract:People with Rett syndrome (RTT) have severe communicative difficulties. They have as well an immature brainstem that implies dysfunction of the autonomic nervous system. Music plays an important role in their life, is often used as a motivating tool in a variety of situations and activities, and caregivers are often clear about people with RTTs favourites. The aim of this study was to investigate physiological and emotional responses related to six different musical stimuli in people with RTT. The study included 29 participants with RTT who were referred to the Swedish Rett Center for medical brainstem assessment during the period 2006–2007. 11 children with a typical developmental pattern were used as comparison. A repeated measures design was used, and physiological data were collected from a neurophysiological brainstem assessment. The continuous dependent variables measured were Cardiac Vagal Tone (CVT), Cardiac Sensitivity to Baroreflex (CSB), Mean Arterial Blood Pressure (MAP) and the Coefficient of Variation of Mean Arterial Blood Pressure (MAP-CV). These parameters were used to categorise brainstem responses as parasympathetic (calming) response, sympathetic (activating) response, arousal (alerting) response and unclear response. The results showed that all participants responded to the musical stimuli, but not always in the expected way. It was noticeable that both people with and without RTT responded with an arousal to all musical stimuli to begin with. Even though the initial expressions sometimes changed after some time due to poor control functions of their brainstem, the present results are consistent with the possibility that the RTT participants’ normal responses to music are intact. These findings may explain why music is so important for individuals with RTT throughout life.
Keywords:Rett syndrome (RTT)  Brainstem assessment  Autonomic nervous system  Music  Vibroacoustic stimulation  Emotions  Facial expressions (FACS)
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