Working memory impairment in people with Williams syndrome: Effects of delay, task and stimuli

Williams syndrome (WS) is a neurodevelopmental disorder associated with impaired visuospatial representations subserved by the dorsal stream and relatively strong object recognition abilities subserved by the ventral stream. There is conflicting evidence on whether this uneven pattern in WS extends to working memory (WM). The present studies provide a new perspective, testing WM for a single stimulus using a delayed recognition paradigm in individuals with WS and typically developing children matched for mental age (MA matches). In three experiments, participants judged whether a second stimulus ‘matched’ an initial sample, either in location or identity. We first examined memory for faces, houses and locations using a 5 s delay (Experiment 1) and a 2 s delay (Experiment 2). We then tested memory for human faces, houses, cat faces, and shoes with a 2 s delay using a new set of stimuli that were better controlled for expression, hairline and orientation (Experiment 3). With the 5 s delay (Experiment 1), the WS group was impaired overall compared to MA matches. While participants with WS tended to perform more poorly than MA matches with the 2 s delay, they also exhibited an uneven profile compared to MA matches. Face recognition was relatively preserved in WS with friendly faces (Experiment 2) but not when the faces had a neutral expression and were less natural looking (Experiment 3). Experiment 3 indicated that memory for object identity was relatively stronger than memory for location in WS. These findings reveal an overall WM impairment in WS that can be overcome under some conditions. Abnormalities in the parietal lobe/dorsal stream in WS may damage not only the representation of spatial location but may also impact WM for visual stimuli more generally.     

Affect, Social Behavior, and the Brain in Williams Syndrome

ABSTRACT— Williams syndrome (WS) is a rare genetic disorder characterized by intellectual impairment and a distinctive physical and neuropsychological profile. Relative to their level of intellectual functioning, individuals with WS exhibit strengths in language and face recognition, with deficits in visual-spatial cognition. A heightened appetitive drive toward social interaction is a strong behavioral feature. Relative to other neurodevelopmental disorders, WS has a clearly defined genetic basis, together with a consistent neurocognitive profile of strengths and deficits. Thus, this disorder offers unique opportunities for elucidating gene–brain–behavior relationships. We focus on manifestations of the unusual social profile in WS, by examining data within and across levels of cognition, brain, and molecular genetics.     

Marriage and Family Therapists’ Ability to Diagnose Asperger’s Syndrome: A Vignette Study

The purpose of this study was to determine the degree to which AAMFT clinical members can accurately diagnose Asperger’s Syndrome. 171 AAMFT clinical members were surveyed using a vignette methodology. Participants were asked to read the vignette and provide a diagnosis for the child in the vignette. Four out of five participants were unable to correctly diagnose the vignette child with Asperger’s Syndrome. Analyses found that the ability to correctly diagnose Asperger’s Syndrome did not vary based on the participants: gender, years of clinical experience, or specialized clinical population (e.g., individuals, couples, families, children). Thomas Stone Carlson is an associate professor and Christi McGeorge is an assistant professor in the couple and family therapy program at North Dakota State University. Sarah Halvorson is a graduate of the couple and family therapy program at North Dakota State University and is in clinical practice in Illinois.     

The theoretical diagnosis of skepticism

Radical skepticism about the external implies that no belief about the external is even prima facie justified. A theoretical reply to skepticism has four stages. First, show which theories of epistemic justification support skeptical doubts (show which theories, given other reasonable assumptions, entail skepticism). Second, show which theories undermine skeptical doubts (show which theories, given other reasonable assumptions, do not support the skeptic’s conclusion). Third, show which of the latter theories (which non-skeptical theory) is correct, and in so doing show that all of the rival theories of justification, skeptical and non-skeptical alike, are mistaken. Fourth, explain why skeptical doubts are sometimes (or sometimes merely seem) intuitive, and thereby accommodate skeptical doubts without capitulation. Michael Williams has pioneered the very idea of a theoretical reply. A theoretical diagnosis consists in just the first two stages. An adequate reply, which is correct at each stage, would rebut the skeptic entirely. Williams’ own reply, I argue, is inadequate. I offer in its place an exhaustive and accurate diagnosis of skepticism. I distinguish four kinds of skepticism and five theories of justification. I then show which theories do, and which theories do not, support which kinds of skepticism.     

Family Impact of Tourette's Syndrome

Tourette's syndrome (TS) is a neuropsychological disorder characterized by vocal and motor tics. TS is also associated with several behavior disorders such as Attention Deficit Hyperactivity Disorder, Oppositional Defiant Disorder, conduct disorder, and Obsessive-Compulsive Disorder. We examined the impact of Tourette's syndrome with and without comorbid psychiatric disorders on the family. TS complicated by comorbid disorders had a greater impact on the family than uncomplicated TS. Tourette's symptom severity was significantly correlated with the level of impact on the family and with the number of comorbid disorders. TS is a disorder with effects that extend beyond motor and vocal tics.     

The ‘Constitutive Thought’ of Regret

In this paper I defend and develop Bernard Williams’ claim that the ‘constitutive thought’ of regret is ‘something like “how much better if it had been otherwise”’. An introductory section on cognitivist theories of emotion is followed by a detailed investigation of the concept of ‘agent-regret’ and of the ways in which the ‘constitutive thought’ might be articulated in different situations in which agents acknowledge casual responsibility for bringing about undesirable outcomes. Among problematic cases discussed are those in which agents have caused harm through no fault of their own, or have been constrained to choose the lesser of two evils or to act against their moral values. R. Jay Wallace’s ‘bourgeois predicament’ and related cases, in which we recognize that our present advantages have flowed from regrettable antecedents, further show that regret is often not a simple emotion, and it is argued that conflicted regrets are sometimes unavoidable. Finally, the paper looks at Descartes’ account of regret as a form of sadness engendered by the recollection of irrecoverable happy experiences, to which the ‘constitutive thought’ does not readily apply. It is suggested that what Descartes is discussing is a different genre of emotion for which ‘nostalgia’ might be a better name.     

What makes counting count? Verbal and visuo-spatial contributions to typical and atypical number development

Williams Syndrome (WS) is marked by a relative strength in verbal cognition coupled with a serious impairment in non-verbal cognition. A strong deficit in numerical cognition has been anecdotally reported in this disorder; however, its nature has not been systematically investigated. Here, we tested 14 children with WS (mean age=7 years 2 months), 14 typically developing controls individually matched on visuo-spatial ability (mean age=3 years 5 months) as well as a larger group of typically developing controls (mean age=3 years 4 months) on two tasks to assess their understanding that counting determines the exact quantity of sets (cardinality principle). The understanding of the cardinality principle in children with WS is extremely delayed and only at the level predicted by their visuo-spatial MA. In this clinical group, only language accounted for a significant amount of the variance in cardinality understanding, whereas in the normal comparison group only visuo-spatial competence predicted the variance. The present findings suggest that visuo-spatial ability plays a greater role than language ability in the actual development of cardinality understanding in typically developing children, whereas the opposite obtains for the clinical group.     

Conservatism in Metaethics: A Case Study

Metaethicists typically develop and assess their theories—in part—on the basis of the consistency of those theories with “ordinary” first‐order normative judgment. They are, in this sense, “methodologically conservative.” This article shows that this methodologically conservative approach obstructs a proper assessment of the debate between internalists and externalists. Specifically, it obstructs one of the most promising readings of internalism. This is a reading—owed to Bernard Williams—in which internalism is part of a practically and politically motivated revision of the assessment of action. The article uses this case study to highlight the role of methodological conservatism in contemporary metaethics more generally.     

Anomalous experiences and schizotypy: which comes first? The Hermit Crab syndrome hypothesis

This study confirms findings of previous research as they state that some triggering events or anomalous experiences can give rise to a disruption of cognitive processes and emotional changes in a predisposed person. Furthermore, our research states that the cognitive process and change of world view (paradigm shift (PS)) accompanying some paranormal experiences (PEs) could facilitate the development of a schizotypal personality structure. For the purposes of this study, 675 young people aged between 13 and 25 years old (M?=?16.8, SD?=?1.9) completed the Schizotypal Personality Questionnaire, the Revised and Modified Paranormal Belief Scales, the measurement of eight kinds of PE and a self-reported measurement of change of world view. The results confirm the mediating role of PS between paranormal practices and schizotypy as the cognitive process contributing to symptom formation as well as to symptom maintenance. The said results also confirm the existence of cognitive–emotional stages, also called Hermit Crab syndrome, in the process of the “paranormalisation” of reality.