Background: People with profound and multiple disabilities often display aberrant behaviours (such as stereotypical movements) which can inhibit learning and lead to social stigmatisation. This study was designed to help a man with multiple disabilities increase his object-contact responses and reduce hand stereotypy (in this case, hand/wrist rotation).
Method: An A-B-A-B design with a 3-month post-intervention check was employed. At baseline, a combination of microswitches (or microswitch cluster) was used to monitor the man's object-contact responses and hand stereotypy. During the intervention phases and post-intervention check, the microswitch cluster was combined with preferred stimuli for object-contact responses occurring without hand stereotypy. The stimulation lasted for 8 seconds if the man remained free from hand stereotypy for that time, but was interrupted if the stereotypy appeared.
Results: The intervention showed an increase in object-contact responses occurring without hand stereotypy and a reduction in session time with hand stereotypy. These data were maintained at the 3-month post-intervention check.
Conclusion: Replication of these findings with other individuals would confirm the utility of this type of intervention for increasing constructive responding and reducing stereotyped behaviours.
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http://dx.doi.org/10.1080/13668250802441664 | DOI Listing |
Ann Clin Transl Neurol
January 2025
Section of Pediatric Neurology and Developmental Neuroscience, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, 77030, USA.
Objective: Rett syndrome (RTT) and MECP2 duplication syndrome (MDS) result from under- and overexpression of MECP2, respectively. Preclinical studies using genetic-based treatment showed robust phenotype recovery for both MDS and RTT. However, there is a risk of converting MDS to RTT, or vice versa, if accurate MeCP2 levels are not achieved.
View Article and Find Full Text PDFPLoS One
November 2024
Faculté des Sciences, Laboratoire de Morphologie Fonctionnelle et Evolutive, Universiteé de Lieège, Lieège, Belgium.
The literature on sound production behaviours in fish in the wild is quite sparse. In several taxa, associations between different sound types and given behaviours have been reported. In the Holocentridae, past nomenclature of the different sound types (knocks, growls, grunts, staccatos and thumps) has been confusing because it relies on the use of several terms that are not always based on fine descriptions.
View Article and Find Full Text PDFNat Rev Dis Primers
November 2024
Department of Neurology, Boston Children's Hospital, Boston, MA, USA.
Rett syndrome (RTT) is a severe, progressive, neurodevelopmental disorder, which affects predominantly females. In most cases, RTT is associated with pathogenic variants in MECP2. MeCP2, the protein product of MECP2, is known to regulate gene expression and is highly expressed in the brain.
View Article and Find Full Text PDFJ Neurodev Disord
October 2024
Centre for Child Health Research, The Kids Research Institute Australia, University of Western Australia, 15 Hospital Avenue, Nedlands, WA, 6009, Australia.
Introduction: Rett syndrome (RTT) is a rare neurodevelopmental disorder with developmental impairments, comorbidities, and abnormal behaviours such as hand stereotypies and emotional features. The Rett Syndrome Behaviour Questionnaire (RSBQ) was developed to describe the behavioural and emotional features of RTT. Little is known how RSBQ scores are associated with genetic and clinical characteristics in RTT.
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