Towards Scientific Incident Response

A scientific incident analysis is one with a methodical, justifiable approach to the human decision-making process. Incident analysis is a good target for additional rigor because it is the most human-intensive part of incident response. Our goal is to provide the tools necessary for specifying precisely the reasoning process in incident analysis. Such tools are lacking, and are a necessary (though not sufficient) component of a more scientific analysis process. To reach this goal, we adapt tools from program verification that can capture and test abductive reasoning. As Charles Peirce coined the term in 1900, “Abduction is the process of forming an explanatory hypothesis. It is the only logical operation which introduces any new idea.” We reference canonical examples as paradigms of decision-making during analysis. With these examples in mind, we design a logic capable of expressing decision-making during incident analysis. The result is that we can express, in machine-readable and precise language, the abductive hypotheses than an analyst makes, and the results of evaluating them. This result is beneficial because it opens up the opportunity of genuinely comparing analyst processes without revealing sensitive system details, as well as opening an opportunity towards improved decision-support via limited automation

The pathophysiology of restricted repetitive behavior

Restricted, repetitive behaviors (RRBs) are heterogeneous ranging from stereotypic body movements to rituals to restricted interests. RRBs are most strongly associated with autism but occur in a number of other clinical disorders as well as in typical development. There does not seem to be a category of RRB that is unique or specific to autism and RRB does not seem to be robustly correlated with specific cognitive, sensory or motor abnormalities in autism. Despite its clinical significance, little is known about the pathophysiology of RRB. Both clinical and animal models studies link repetitive behaviors to genetic mutations and a number of specific genetic syndromes have RRBs as part of the clinical phenotype. Genetic risk factors may interact with experiential factors resulting in the extremes in repetitive behavior phenotypic expression that characterize autism. Few studies of individuals with autism have correlated MRI findings and RRBs and no attempt has been made to associate RRB and post-mortem tissue findings. Available clinical and animal models data indicate functional and structural alterations in cortical-basal ganglia circuitry in the expression of RRB, however. Our own studies point to reduced activity of the indirect basal ganglia pathway being associated with high levels of repetitive behavior in an animal model. These findings, if generalizable, suggest specific therapeutic targets. These, and perhaps other, perturbations to cortical basal ganglia circuitry are mediated by specific molecular mechanisms (e.g., altered gene expression) that result in long-term, experience-dependent neuroadaptations that initiate and maintain repetitive behavior. A great deal more research is needed to uncover such mechanisms. Work in areas such as substance abuse, OCD, Tourette syndrome, Parkinson’s disease, and dementias promise to provide findings critical for identifying neurobiological mechanisms relevant to RRB in autism. Moreover, basic research in areas such as birdsong, habit formation, and procedural learning may provide additional, much needed clues. Understanding the pathophysioloy of repetitive behavior will be critical to identifying novel therapeutic targets and strategies for individuals with autism

Exploratory Study of Executive Function Abilities Across the Adult Lifespan in Individuals Receiving an ASD Diagnosis in Adulthood

The few studies of autism spectrum disorder (ASD) across adulthood suggest different age-related associations in different aspects of executive function (EF). In this exploratory study we examined EF abilities and self-report autism traits in 134 adults (aged 18-75 years; mean=31 years) with abilities in the normal range, receiving a first diagnosis of ASD. Results suggest that in some EF relying on speed and sequencing (Trails A and B; Digit Symbol), late-diagnosed ASD individuals may demonstrate better performance than typical age-norms. On other EF (Digit Span, Hayling, Brixton tests) age-related correlations were similar to typical age-norms. Different domains of EF may demonstrate different trajectories for ageing with ASD, with patterns of slower, accelerated or equivalent age-related change observed across different measures

Exploratory Study of Executive Function Abilities Across the Adult Lifespan in Individuals Receiving an ASD Diagnosis in Adulthood

The few studies of autism spectrum disorder (ASD) across adulthood suggest different age-related associations in different aspects of executive function (EF). In this exploratory study we examined EF abilities and self-report autism traits in 134 adults (aged 18-75 years; mean=31 years) with abilities in the normal range, receiving a first diagnosis of ASD. Results suggest that in some EF relying on speed and sequencing (Trails A and B; Digit Symbol), late-diagnosed ASD individuals may demonstrate better performance than typical age-norms. On other EF (Digit Span, Hayling, Brixton tests) age-related correlations were similar to typical age-norms. Different domains of EF may demonstrate different trajectories for ageing with ASD, with patterns of slower, accelerated or equivalent age-related change observed across different measures

Williams Syndrome and memory: a neuroanatomic and cognitive approach

Williams Syndrome (WS) is described as displaying a dissociation within memory systems. As the integrity of hippocampal formation (HF) is determinant for memory performance, we examined HF volumes and its association with memory measures in a group of WS and in a typically development group. A significantly reduced intracranial content was found in WS, despite no differences were observed for HF absolute volumes between groups. When volumes were normalized, left HF was increased in WS. Moreover, a lack of the normal right[left HF asymmetry was observed in WS. No positive correlations were found between volumetric and neurocognitive data in WS. In sum, a relative enlargement of HF and atypical patterns of asymmetry suggest abnormal brain development in WSThis research was supported by the grants PIC/IC/83290/2007 from Fundação para a Ciência e Tecnologia (Portugal). This study was also supported, in part, by grants from the National Institutes of Health (K05 MH 01110)