Applying Cluster Analysis to Define a Typology of Chronic Fatigue Syndrome in a Medically-Evaluated,Random Community Sample

This study involved a randomly selected, medically-evaluated, community-based sample of 166 individuals with chronic fatigue. Participants diagnosed with chronic fatigue syndrome and medically-explained chronic fatigue reported significantly more severe fatigue following exertion than the idiopathic chronic fatigue group, and participants with medically-explained chronic fatigue also reported significantly more severe fatigue following exertion than the psychiatrically-explained chronic fatigue group. A cluster analysis was performed to define a typology of chronic fatigue symptomatology for participants diagnosed with chronic fatigue syndrome. Three clusters emerged. Cluster 1 contained only one participant with chronic fatigue syndrome and was characterized by relatively low post-exertional fatigue. Cluster 2 contained a small proportion of individuals with chronic fatigue syndrome and was characterized by most severe post-exertional fatigue and most improvement in fatigue following rest. Cluster 3 contained the highest proportion of individuals with chronic fatigue syndrome, and was characterized by high post-exertional fatigue and fatigue not alleviated by rest.     

22q11.2 Deletion Syndrome: Genetics,Neuroanatomy and Cognitive/Behavioral Features Keywords

This paper presents a conceptual review of the genetic underpinnings of 22q11.2 Deletion Syndrome. The neuroanatomical, neuropsychological, behavioral, and psychiatric phenotype associated with 22q11.2 Deletion Syndrome is also explored, including variables that are thought to affect symptom expression. The history of the deletion syndrome is described, and future directions for continued research are discussed.     

Expression des états mentaux chez les enfants atteints du Syndrome de Williams : analyse d'interactions collaboratives mère-enfant

People affected by Williams syndrome (WS), a rare genetic disease, are characterized by a personality noted for its “hypersociability” along with relatively spared language in the face of significant cognitive deficit. Our research focuses on the intersection of two domains by examining linguistic markers of this sociability: how do children with WS perform in a collaborative interaction? Do they express mental states, how and what kind? And do they answer to the mental states expressed by others? We present data from three matched groups of 12 each: children with WS, children with Down syndrome (DS) and control group (CO) in a task in which the mother and child interacted in a standardized collaborative situation with a social stake (a precise aim to reach). The children with WS had comparable difficulties to those with DS in the interaction: they produced fewer turns and fewer utterances than controls. Children with WS produced more expressive mental states than either the DS or CO group. The WS children had more difficulties cooperating than did either the DS or CO children: the unrealized requests by the children are more numerous in the WS group. Thus in a collaborative interaction, the children with WS present a unique profile: difficulty in maintaining and participating in the interaction contrasting with a facility in expressing mental states about feelings and emotions.     

Dynamic balance assessment during gait in children with Down and Prader-Willi syndromes using inertial sensors

Down (DS) and Prader-Willi (PWS) syndromes are chromosomal disorders both characterized by obesity, ligament laxity, and hypotonia, the latter associated with gait instability. Although these shared features may justify a common rehabilitation approach, evidence exists that adults with DS and PWS adopt different postural and walking strategies. The development of an instrumented protocol able to describe these strategies and quantify patients’ gait stability in the current clinical routine would be of great benefit for health professionals, allowing them to design personalized rehabilitation programs. This is particularly true for children with DS and PWS, where motor development is dramatically constrained by severe hypotonia and muscle weakness. The aim of this study was, thus, to propose an instrumented protocol, integrated with the clinical routine and based on the use of wearable inertial sensors, to assess gait stability in DS and PWS children.Fifteen children with DS, 11 children with PWS, and 12 typically developing children (CG) were involved in the study. Participants performed a 10-meter walking test while wearing four inertial sensors located at pelvis, sternum, and both distal tibiae levels. Spatiotemporal parameters (walking speed, stride frequency, and stride length) and a set of indices related to gait symmetry and upper-body stability (Root Mean Square, Attenuation Coefficient and Improved Harmonic Ratio) were estimated from pelvis and sternum accelerations. The Gross Motor Functional Measures (GMFM-88) and Intelligence Quotient (IQ Wechsler) were also assessed for each patient. A correlation analysis among the GMFM-88 and IQ scales and the estimated parameters was then performed.Children with DS and PWS exhibit reduced gait symmetry and higher accelerations at pelvis level than CG. While these accelerations are attenuated by about 40% at sternum level in CG and DS, PWS children display significant smaller attenuations, thus reporting reduced gait stability, most likely due to their typical “Trendelenburg gait”. Significant correlations were found between the estimated parameters and the GMFM-88 scale when considering the whole PWS and DS group and the PWS group alone.These results promote the adoption of wearable technology in clinical routines to monitor gait patterns in children with DS and PWS: the proposed protocol allows to markedly characterize patient-specific motor limitations even when clinical assessment scores provide similar results in terms of pathology severity. This protocol could be adopted to support health professionals in designing personalized treatments that, in turn, could help improving patients’ quality of life in terms of both physical and social perspectives.     

心理理论视角下的Asperger综合症、精神分裂症和孤独症

心理理论是儿童社会认知发展研究的重要课题,文章对心理理论研究在临床实践中的应用进行综述,详细介绍了Asperger综合症,精神分裂症,孤独症（自闭症）中心理理论的研究,并对以后的研究提出建议。     

Prevalence and treatment of Chronic Fatigue Syndrome/Myalgic Encephalomyelitis and co-morbid severe health anxiety

Chronic Fatigue Syndrome/Myalgic Encephalomyelitis (CFS/ME) is a debilitating condition that affects 0.2–0.4% of the population. Health focussed anxiety is common across medical conditions, and may be relevant in CFS/ME. This study sought to identify the prevalence and impact of health anxiety (HA) in CFS/ME and evaluate the effectiveness of Cognitive Behavioural Therapy for HA in CFS/ME. Cross-sectional questionnaire methods and case-series design were used to achieve study aims. Analysis indicated that 41.9% of the CFS/ME clinic sample experienced threshold levels of health anxiety, which was associated with elevated symptom severity across several dimensions. Stepwise multiple regression indicated physical functioning and depression accounted for 23.8% of variance in fatigue; depression, fatigue and HA, accounted for 32.9% of variance in physical functioning. Large effect sizes and clinically significant changes were generated in the treatment study. HA is common in CFS/ME and likely to exacerbate fatigue and physical functioning. This study identifies HA as an important target for treatment, trial findings should be further replicated on a larger scale.