A cross-syndrome study of the development of holistic face recognition in children with autism, Down syndrome, and Williams syndrome

We report a cross-syndrome comparison of the development of holistic processing in face recognition in school-aged children with developmental disorders: autism, Down syndrome, and Williams syndrome. The autism group was split into two groups: one with high-functioning children and one with low-functioning children. The latter group has rarely been studied in this context. The four disorder groups were compared with typically developing children. Cross-sectional trajectory analyses were used to compare development in a modified version of Tanaka and Farah’s part–whole task. Trajectories were constructed linking part–whole performance either to chronological age or to several measures of mental age (receptive vocabulary, visuospatial construction, and the Benton Facial Recognition Test). In addition to variable delays in onset and rate of development, we found an atypical profile in all disorder groups. These profiles were atypical in different ways, indicating multiple pathways to, and variable outcomes in, the development of face recognition. We discuss the implications for theories of face recognition in both atypical and typical development, including the idea that part–whole and rotation manipulations may tap different aspects of holistic and/or configural processing.     

Differences in alexithymia and emotional awareness in exhaustion syndrome and chronic fatigue syndrome

Symptoms of Exhaustion Syndrome (ES) and Chronic Fatigue Syndrome (CFS) are overlapping and create difficulties of differential diagnosis. Empirical studies comparing ES and CFS are scarce. This study aims to investigate if there are any emotional differences between ES and CFS. This cross‐sectional study compared self‐reported alexithymia and observer‐rated emotional awareness in patients with ES (n = 31), CFS (n = 38) and healthy controls (HC) (n = 30). Self‐reported alexithymia was measured with the Toronto Alexithymia Scale‐20 (TAS‐20) and emotional awareness with an observer‐rated performance test, the Level of Emotional Awareness Scale (LEAS). Additionally, depression and anxiety were scored by the Hospital Anxiety and Depression Scale (HADS). Results show that patients with ES expressed higher self‐reported alexithymia in the TAS‐20 compared to HC, but had similar emotional awareness capacity in the observer‐rated performance test, the LEAS. Patients with CFS expressed more difficulties in identifying emotions compared to HCs, and performed significantly worse in the LEAS‐total and spent more time completing the LEAS as compared to HC. Correlation and multiple regressions analyses revealed that depression and anxiety positively correlated with and explained part of the variances in alexithymia scores, while age and group explained the major part of the variance in LEAS. Findings of this study indicate that emotional status is different in patients with ES and CFS with respect to both self‐reported alexithymia and observer‐rated emotional awareness. Emotional parameters should be approached both in clinical investigation and psychotherapy for patients with ES and CFS.     

Facial emotion recognition in Williams syndrome and Down syndrome: A matching and developmental study

In this study both the matching and developmental trajectories approaches were used to clarify questions that remain open in the literature on facial emotion recognition in Williams syndrome (WS) and Down syndrome (DS). The matching approach showed that individuals with WS or DS exhibit neither proficiency for the expression of happiness nor specific impairments for negative emotions. Instead, they present the same pattern of emotion recognition as typically developing (TD) individuals. Thus, the better performance on the recognition of positive compared to negative emotions usually reported in WS and DS is not specific of these populations but seems to represent a typical pattern. Prior studies based on the matching approach suggested that the development of facial emotion recognition is delayed in WS and atypical in DS. Nevertheless, and even though performance levels were lower in DS than in WS, the developmental trajectories approach used in this study evidenced that not only individuals with DS but also those with WS present atypical development in facial emotion recognition. Unlike in the TD participants, where developmental changes were observed along with age, in the WS and DS groups, the development of facial emotion recognition was static. Both individuals with WS and those with DS reached an early maximum developmental level due to cognitive constraints.     

Personality traits in women with Turner syndrome

Turner syndrome is caused by the total or partial absence of, or structural abnormalities in, one sex chromosome. A personality style characterized by traits related to low neuroticism and high extroversion has been reported for females with Turner syndrome, indicating possible genetic factors related to personality development. This study examines personality traits in 37 Swedish women with Turner syndrome using the Karolinska Scales of Personality Inventory. Compared to reference data, the subjects rated themselves as more sensation seeking, less detached, less verbally aggressive and less irritated. These results partially support previous findings. The subjects were divided into three karyotype groups: 45,X (n = 13), 45,X/46,XX or 45,X/45,XY (n = 13), and structural abnormalities (n = 11). Contrary to some previous findings, no differences in personality were found between the karyotype groups.     

Impaired tactual perception in children with Down's syndrome

Using standardized neuropsychological tests, tactual finger discrimination, graphaesthesia and stereognosis were studied in a group of 11 children aged 7–11 years with Down's syndrome (DS). All tasks were solved without visual control. The performance was subnormal in each test and the results of the stereognosis test were particularly poor. It was ascertained that the DS children could understand test instructions. Moreover, under visual control all objects used in the stereognosis test were handled correctly. The results suggest that tactual perception is subnormal in children with DS. The pathogenesis of the somatosensory abnormalities should be explored.     

The cognitive profile of Sotos syndrome

Sotos syndrome is a congenital overgrowth disorder, associated with intellectual disability. Previous research suggests that Sotos syndrome may be associated with relative strength in verbal ability and relative weakness in non-verbal reasoning ability but this has not been explicitly assessed. To date, the cognitive profile of Sotos syndrome is unknown. Cognitive abilities of a large and representative sample of individuals with Sotos syndrome (N = 52) were assessed using the British Ability Scales (BAS3). The majority of participants had intellectual disability or borderline intellectual functioning. The cluster score profile analysis revealed a consistent verbal ability > non-verbal reasoning ability profile. Four specific criteria were proposed as the Sotos syndrome cognitive profile (SSCP): verbal ability > non-verbal reasoning ability; quantitative reasoning T-score or matrices T-score <20th percentile; quantitative reasoning T-score < mean T-score; recognition of designs T-score or recognition of pictures T-score > mean T-score. Of the 35 participants included in the profile analysis, 28 met all four SSCP criteria, yielding a sensitivity of 0.8. The sensitivity of each of the SSCP criteria was >0.9. Individuals with Sotos syndrome display a clear and consistent cognitive profile, characterized by relative strength in verbal ability and visuospatial memory but relative weakness in non-verbal reasoning ability and quantitative reasoning. This has important implications for the education of individuals with Sotos syndrome.     

People with Williams syndrome process faces holistically

This study compared the performance of 47 adolescents and adults with Williams syndrome to 39 age-matched controls on a face recognition task. Using the whole-part paradigm developed by Tanaka and his colleagues, we found that although performance overall was lower in the participants with Williams syndrome, both groups showed similar patterns of performance across the different conditions. Both groups performed significantly better in the whole-face than in the isolated-part test condition for upright faces, but not for inverted faces. The whole-face advantage only in the upright condition provides strong evidence that people with Williams syndrome encode and recognize faces holistically in the same way as normal controls, suggesting the use of similar underlying neurocognitive mechanisms. These findings contradict earlier reports in the literature that people with Williams syndrome process faces abnormally.     

Cognitive therapy for irritable bowel syndrome is associated with reduced limbic activity, GI symptoms, and anxiety

This study sought to identify brain regions that underlie symptom changes in severely affected IBS patients undergoing cognitive therapy (CT). Five healthy controls and 6 Rome II diagnosed IBS patients underwent psychological testing followed by rectal balloon distention while brain neural activity was measured with O-15 water positron emission tomography (PET) before and after a brief regimen of CT. Pre-treatment resting state scans, without distention, were compared to post-treatment scans using statistical parametric mapping (SPM). Neural activity in the parahippocampal gyrus and inferior portion of the right cortex cingulate were reduced in the post-treatment scan, compared to pre-treatment (x, y, z coordinates in MNI standard space were -30, -12, -30, P=0.017; 6, 34, -8, P=0.023, respectively). Blood flow values at these two sites in the controls were intermediate between those in the pre- and post-treatment IBS patients. Limbic activity changes were accompanied by significant improvements in GI symptoms (e.g., pain, bowel dysfunction) and psychological functioning (e.g., anxiety, worry). The left pons (-2, -26, -28, P=0.04) showed decreased neural activity which was correlated with post-treatment anxiety scores. Changes in neural activity of cortical-limbic regions that subserve hypervigilance and emotion regulation may represent biologically oriented change mechanisms that mediate symptom improvement of CT for IBS.     

Neurocognitive functioning in chronic fatigue syndrome

Although substantial research has been conducted on chronic fatigue syndrome (CFS) over the past decade, the syndrome remains poorly understood. The most recent case definition describes CFS as being characterized both by disabling fatigue and by subjective reports of difficulty with concentration and short-term memory. However, research into the neurocognitive and psychological functioning of individuals with CFS has provided mixed objective results. The current paper reviews studies that have examined the neurocognitive and/or psychological functioning of individuals with CFS. Changes in research design and instruments employed to study individuals with CFS are suggested.     

A study of Ghiselli's hobo syndrome

This study attempts to clarify conceptual and operational inconsistencies in the literature around “Ghiselli's hobo syndrome.” I propose that defining characteristics of hobo syndrome should include both the exhibition of frequent job movement behavior and positive attitudes about such behavior. This definition effectively differentiates the construct from other similar phenomena associated with frequent job movement (e.g., job/career mobility, protean careers). Using latent class cluster analysis of a diverse sample of 944 U.S. workers, it was empirically validated that a small number of individuals resembling the proposed characteristics of hobos did emerge as a distinct group (N = 42), providing person-centered evidence for the construct validity of hobo syndrome. The dispositional roots of hobo syndrome and work-related outcomes were also explored. It was found that individuals with high levels of openness to experience were likely to exhibit hobo syndrome, whereas impulsivity was not related to such tendency. Also, workers who were more likely to be categorized as hobos tended to report less positive views about their current jobs. Based on these findings, implications for research and practice are discussed.     

Gender differences in irritable bowel syndrome among medical students at Inner Mongolia Medical University,China: a cross-sectional study

There is little epidemiological research on Irritable bowel syndrome (IBS) in Inner Mongolia, China. Here we investigated the prevalence of IBS and factors associated with IBS in both males and females in Inner Mongolia Medical University by a cross-sectional study. We recruited Inner Mongolia Medical University students residing in campus and asked them to complete a self-administered questionnaire. The prevalence of IBS in each factor we chose in all, male, and female students was determined. We assessed IBS subtypes in male and female students. Multivariate logistic regression models were used to assess the factors associated with IBS in male and female students. The overall prevalence of IBS was 29.5%. The prevalence of IBS in female students was significantly higher than that in male students (31.3% vs. 24.8%, p < .001). Logistic regression results showed that attempting to lose weight and anxiety were both associated with increasing odds of IBS, while exercise was not associated with IBS in either male or female students. In female students, snack consumption and depression were also both associated with increasing odds of IBS. The predominant IBS subtype was the diarrhea-predominant type in both male and female students. Considering the high prevalence of IBS in students and the fact that the factors associated with IBS can be improved by individuals, students should be given adequate education and counseling to improve their mental health and lifestyle, especially female students in higher grades.     

Differences in visual orienting between persons with Down or fragile X syndrome

The voluntary and reflexive orienting abilities of persons with Down syndrome and fragile X syndrome, at average MA levels of approximately 4 and 7 years, were compared with an RT task. Reflexive orienting abilities appeared to develop in accordance with MA for the participants with Down syndrome but not for those with fragile X syndrome. However, both groups showed delayed voluntary orienting. The group differences in reflexive orienting at the low MA level reinforce the practice of separating etiologies and highlight the contribution of rudimentary attentional processes in the study of individuals with mental retardation.     

Neural correlates of music recognition in Down syndrome

The brain mechanisms that subserve music recognition remain unclear despite increasing interest in this process. Here we report the results of a magnetoencephalography experiment to determine the temporal dynamics and spatial distribution of brain regions activated during listening to a familiar and unfamiliar instrumental melody in control adults and adults with Down syndrome (DS). In the control group, listening to the familiar melody relative to the unfamiliar melody, revealed early and significant activations in the left primary auditory cortex, followed by activity in the limbic and sensory-motor regions and finally, activation in the motor related areas. In the DS group, listening to the familiar melody relative to the unfamiliar melody revealed increased significant activations in only three regions. Activity began in the left primary auditory cortex and the superior temporal gyrus and was followed by enhanced activity in the right precentral gyrus. These data suggest that familiar music is associated with auditory–motor coupling but does not activate brain areas involved in emotional processing in DS. These findings reveal new insights on the neural basis of music perception in DS as well as the temporal course of neural activity in control adults.     

The child's experience of Munchhausen syndrome by proxy

This paper examines the emotional impact of Munchhausen syndrome by proxy on a child aged 6 who had been poisoned by her mother. It describes her treatment in child psychotherapy over twenty months at the Cassel Hospital where the family worked towards her successful rehabilitation home. The work with her showed her experience of dangerous relationships in her family that left her distrustful of any new relationship, and doubtful of her capacity to be close without being destructive. Like her parents, she was terrified of facing the murderous feelings in the family, retreating into denial when life was too painful. The paper describes three phases of treatment: fear of separation and preoccupation with the trauma of poisoning, ambivalence about facing the destructiveness in the family and the move into more appropriate latency behaviour as she prepared to go home with her parents. It concludes with an overview of the impact of Munchhausen syndrome by proxy on the developing child, as shown in this treatment: the delay in emotional and cognitive development, the interference with the capacity to relate, the retreat into illness under stress, and the fear of permanent damage caused by the emotional and physical abuse.     

Attentional disengagement in adults with Williams syndrome

Williams syndrome (WS) is a neurodevelopmental disorder characterized by a distinctive behavioral and cognitive profile, including widespread problems with attention. However, the specific nature of their attentional difficulties, such as inappropriate attentional allocation and/or poor attentional disengagement abilities, has yet to be elucidated. Furthermore, it is unknown if there is an underlying difficulty with the temporal dynamics of attention in WS or if their attentional difficulties are task-dependent, because previous studies have examined attention in established areas of deficit and atypicality (specifically, visuospatial and face processing). In this study, we examined attentional processing in 14 adults with WS (20–59 years) and 17 typically developing controls (19–39 years) using an attentional blink (AB) paradigm. The AB is the decreased ability to detect a second target when it is presented in close proximity to an initial target. Overall, adults with WS had an AB that was prolonged in duration, but no different in magnitude, compared with typically developing control participants. AB performance was not explained by IQ, working memory, or processing speed in either group. Thus, results suggest that the attention problems in WS are primarily due to general attentional disengagement difficulties rather than inappropriate attentional allocation.     

Historical misrepresentation in science: The case of fetal alcohol syndrome

The history of the fetal alcohol syndrome (FAS) provides a microcosm in which to explore the larger ramifications of historical citations in biomedical publications. Though some historical references such as Biblical writings may hint at a rudimentary understanding of the relationship between maternal drinking and fetal development, no definitive case can be made for an understanding of FAS dating back hundreds of years. Authors who claim an impressive history for FAS misrepresent that history. The modern history of FAS raises a question concerning citations of original discoveries. The first paper describing ethanol-induced damage to the fetus appeared in 1968 yet most researchers cite one of two papers from 1973. Both ancient citations and modern references to original discoveries pose difficult questions for the scientist. Both dilemmas may be solved by a better reading of the literature and a more judicious wording when writing about history. This work was supported in part by Grant # AA10681 to SNP.     

Behavioral and pharmacological treatments for tourette syndrome: A review

This article reviews the published treatment outcome research on pharmacological and behavioral treatments for Gilles de la Tourette syndrome (TS). Controlled group outcome studies of pharmacological treatments show about a 50–60% reduction in tics with haloperidol and pimozide and about a 20% reduction with clonidine. A controlled group outcome study and several within-subject design studies of behavioral treatments show about a 90% reduction in tics with habit reversal training. A large number of case studies generally confirm these results and also show benefits from other behavioral treatments such as relaxation training, self-monitoring, and contingency management. Clinical limitations of TS drugs are that they produce side effects in 50–85% of the patients and require continuous use, and long-term compliance with the medications is limited. The primary limitation of behavioral treatments is that they require a large initial time commitment. The methodological strengths of the controlled drug studies are the use of double-blind and group designs. For the behavioral studies, the strengths are rigorous recording and controlled within-subject designs.