The Changing Age of Individuals Seeking Presymptomatic Genetic Testing for Huntington Disease

Huntington disease (HD) is a progressive neurodegenerative disorder. Presymptomatic genetic testing allows at-risk individuals to clarify their risk status. Understanding the characteristics and motivations of individuals seeking HD presymptomatic genetic testing better equips genetic counselors and other healthcare professionals to provide comprehensive and personalized care. The aims of this study were to (1) determine whether the average age when individuals seek presymptomatic HD genetic testing has decreased over time, (2) assess motivations for seeking testing, (3) explore whether there is a relationship between age and motivations, and (4) explore genetic counselors’ perceptions of the shift in age. Data from the US HD testing centers (N?=?4) were analyzed. A small but statistically significant decrease in age of individuals seeking presymptomatic testing was observed (p?=?0.045). HD community members (N?=?77) were surveyed regarding presymptomatic testing motivations. Younger individuals were more likely than older individuals to cite “To learn whether or not you would develop HD” and “To make choices about further education or a career” compared to older individuals (p?<?0.05). Conversely, older individuals more frequently cited “To give children a better idea of their risk” (p?<?0.002). Sixteen percent of genetic counselors surveyed (6/37) perceived a change in age of testing. All of these respondents had provided HD testing for ten or more years and anecdotally believed the age at testing has decreased over time. Study results help providers personalize counseling based on patient’s age and serve as a starting point for more research into the relationship between age at testing and motivations for testing.     

The Role of ADHD and Negative Emotional Lability in Predicting Changes in Parenting Daily Hassles

The present study examined the extent to which children’s negative emotional lability (measured via ecological momentary assessment—EMA) and ADHD diagnostic status predicted changes in the frequency of daily parenting hassles and the stress resulting from daily hassles at one-week follow-up when controlling for baseline parenting hassles. Parents of 84 children 8–12 years-old (47 with ADHD, 37 without ADHD) completed a measure of parenting daily hassles at baseline and follow-up and participated in EMA assessment protocol ratings of their child’s mood (3-times daily) for one week. Analyses of covariance indicated that parents of children with ADHD reported significantly greater frequency of daily parenting hassles and intensity of parenting stress resulting from daily hassles than parents of children without ADHD at baseline and follow-up. Hierarchical regression analyses suggested that children’s negative emotional lability was a significant predictor of the intensity of parenting stress resulting from daily hassles, but not the frequency of daily parenting hassles. There was also an interaction of ADHD diagnostic status and greater EMA-derived negative emotional lability in the prediction of the frequency of daily parenting hassles due to children’s challenging behaviors. Specifically, greater negative emotional lability predicted more frequent daily parenting hassles due to children’s challenging behaviors among parents of children without ADHD but not among parents of children with ADHD. Overall, this study suggests that children’s negative emotional lability is a significant predictor of aspects of daily parenting hassles across parents of children with and without ADHD.     

A Review of Behavioral Interventions for Anxiety‐Related Behaviors in Lower‐Functioning Individuals with Autism

Anxiety is highly prevalent in persons with autism and affects individuals with higher‐functioning autism (HFA) and lower‐functioning autism (LFA). Although there is emerging support for the efficacy of cognitive‐behavioral therapy (CBT) in treating anxiety in autism, these findings are largely based on HFA samples. Moreover, CBT may be inappropriate for individuals with LFA who may struggle to grasp the cognitive components of the therapy because of their more limited verbal abilities. Behavior analytic interventions may be better suited to treat anxiety in this population; however, the quantity and quality of the extant literature have yet to be reviewed. Toward this end, the extant behavioral intervention literature was reviewed. Seven studies met the inclusion criteria and were evaluated against quality indicators for within‐subject design research. Next, the efficacy of each treatment component was assessed using established criteria for defining empirically supported therapies. Results revealed behavioral interventions to be efficacious, with desensitization and reinforcement emerging as efficacious treatment components, while prompting, modeling, and antianxiety stimuli were established as possibly efficacious. However, given that some of the quality indicators were not met, these results should be interpreted with some caution. Additional research is needed to form a more conclusive evidence base for the treatment of anxiety in individuals with LFA. Copyright © 2016 John Wiley & Sons, Ltd.     

Anticipation of cognitive demand during decision-making

An abundance of evidence indicates that action selection is guided, at least in certain contexts, by anticipation of action outcomes. In one particularly clear demonstration of this principle, Bechara and colleagues, studying a gambling task, observed phasic skin conductance responses just prior to actions associated with a relatively high risk of monetary loss (Bechara et al. in J Neurosci 19:5473–5481, 1999; Bechara et al. in Science 275:1293–1295, 1997; Bechara et al. in Cereb Cortex 6:215–225, 1996). In the present work, we tested for the same effect in a paradigm where choices resulted not in differential monetary outcomes, but in differential requirements for subsequent mental effort. In two experiments, we observed an anticipatory skin conductance response prior to actions resulting in a high level of cognitive demand. This finding indicates that requirements for effortful cognitive control are anticipated during action selection. We argue, based on convergent evidence, that such anticipation may not only trigger preparation; it may also play a direct role in effort-based decision-making.