: Children with intellectual disability are at risk for anxiety disorders involving intense physiological reactions and risky behavioral responses. Interventions have been identified in this field; however, assessment of underlying anxiety is limited and flawed.: We implemented a single-subject case study using differential reinforcement to treat dog phobia in a boy with intellectual disability. We recorded elopement and compliance with goals and measured physiological expressions of stress: galvanic skin response, heart rate variability, temperature, and latency to calm down.: After fifteen therapy sessions, the boy decreased elopement and noncompliance considerably and showed dramatic improvements in emotional self-regulation.: Future research should examine the utility of including biosensing measures in clinical applications and the relationship between physiological measures of anxiety and traditional questionnaires. Children with intellectual disability at risk for anxiety disorders should be tracked longitudinally to examine the effect of interventions on social-emotional well-being and self-regulation.
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http://dx.doi.org/10.1080/17518423.2019.1683909 | DOI Listing |
CNS Drugs
January 2025
Cornwall Intellectual Disability Equitable Research (CIDER), University of Plymouth, Truro, England.
There is a synergistic relationship between epilepsy and intellectual disability (ID), and the approach to managing people with these conditions needs to be holistic. Epilepsy is the main co-morbidity associated with ID, and clinical presentation tends to be complex, associated with higher rates of treatment resistance, multi-morbidity and premature mortality. Despite this relationship, there is limited level 1 evidence to inform treatment choice for this vulnerable population.
View Article and Find Full Text PDFCalcif Tissue Int
January 2025
Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy.
Rett syndrome (RS) is a rare neurodevelopmental disorder primarily caused by mutations in the X-linked methyl-CpG binding protein 2 (MECP2) gene, responsible for encoding MECP2 which plays a pivotal role in regulating gene expression. The neurological and non-neurological manifestations of RS vary widely in severity depending on the specific mutation type. Bone complications, mostly scoliosis but also osteoporosis, hip displacement, and a high rate of fractures, are among the most prevalent non-neurological comorbidities observed in girls with RS.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
The UC Irvine Institute for Memory Impairments and Neurological Disorders (UCI MIND), Irvine, CA, USA.
Background: Individuals with Down syndrome (DS) have an increased genetic risk of developing Alzheimer's disease (AD), with most adults developing AD neuropathology in their 40s. Despite having a low frequency of systemic vascular risk factors such as hypertension and atherosclerosis, adults with DS display cerebrovascular pathology, including microbleeds, microinfarcts, and cerebral amyloid angiopathy. This suggests that blood-brain barrier (BBB) integrity may be compromised allowing the extravasation of blood proteins in the brain parenchyma.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
University of Kansas Medical Center, Kansas City, KS, USA.
Background: Mitochondrial dysfunction and Aβ accumulation are hallmarks of Alzheimer's disease (AD). However, the role of these pathologies in Down Syndrome associated Alzheimer's Disease (DSAD) is unknown. Decades of research describe a relationship between mitochondrial function and Aβ production.
View Article and Find Full Text PDFAlzheimers Dement
December 2024
NYU Grossman School of Medicine, New York, NY, USA.
Background: Down syndrome (DS) is strongly associated with Alzheimer's disease (AD), attributable to APP overexpression, displaying common features with early-onset AD (EOAD) and late-onset AD (LOAD) like Amyloid-β (Aβ) and tau pathology. Here, we evaluated the Aβ plaques proteome of DS, EOAD and LOAD.
Method: We used unbiased localized proteomics to analyze amyloid plaques and the adjacent plaque-devoid tissue ('AD non-plaque') from post-mortem paraffin-embedded tissues in three subtypes of AD (n = 20/group): DS (59.
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