Neuropathology of Rett syndrome

Rett Syndrome is unlike any other pediatric neurologic disease, and its clinical-pathologic correlation can not be defined with standard histology techniques. Based on hypotheses suggested by careful clinical observations, the nervous system of the Rett child has been explored utilizing morphometry, golgi preparations, computerized tomography, magnetic resonance imaging, chemistry, immunocytochemistry, autoradiography, and molecular biologic techniques. From these many perspectives we conclude that Rett syndrome is not a typical degenerative disorder, storage disorder, nor the result of gross malformation, infectious or neoplastic processes. There remain regions of the brain that have not been studied in detail but the available data suggest that the neuropathology of Rett syndrome can be summarized as follows: the Rett brain is small for the age and the height of the patient; it does not become progressively smaller over three to four decades; it has small dendritic trees in pyramidal neurons of layers III and V in selected lobes (frontal, motor, and temporal); it has small neurons with an increased neuronal packing density; it has an immature expression of microtubular protein-2 and cyclooxygenase; it exhibits a changing pattern of neurotransmitter receptors with an apparent reduction in many neurotransmitters, possibly contributing to some symptomatology. A mutation in Mecp2 causes this unique disorder of brain development. Neuronal mosaicism for normal and mutated Mecp2 produces a consistent phenotype in the classic female patient and a small brain with some preserved islands of function, but with an inability to support hand use and speech. This paper summarizes our current observations about neuropathology of Rett syndrome. MRDD Research Reviews 2002;8:72-76.     

Control of saccades in Parkinson's disease

Parkinson's patients (PD) made pro- and antisaccades: In the no-delay condition, the target appeared concurrent with the GO signal. In the delay condition, the target appeared before the signal for movement. Second, we probed spatial working memory in PD. Subjects looked to the remembered locations of sequential targets. In the no-delay prosaccade condition, PD had faster reaction times, made more express saccades, and exhibited hypometria. In the no-delay antisaccade condition, PD had longer reaction times and made more direction errors. In the delay tasks, PD made more direction errors and had more difficulty withholding a movement. PD made more sequencing errors in the spatial working memory task. These findings are consistent with a basal ganglia pathophysiology influencing eye movement processing in the frontal cortex.     

Aging family caregivers: policies and practices

This review examines later life family support for adults with developmental disabilities from a life course perspective that takes into account social trends and changes in service patterns and in attitudes of families. Key issues addressed include: (1) trends affecting family caregiving, (2) health and social outcomes of life-long caregiving, (3) support needs of families, (4) family support policies and practices, and (5) recommendations for a research and policy agenda. Research examining outcomes of life-long caregiving has shown that most families adapt well to having a family member with disabilities. However, some families are at risk for poorer physical and mental health outcomes. These include cultural minorities and families of adults with behavioral challenges. Caregiving does seem to have a negative impact on maternal employment and family income as mothers often give up or cut back on employment to care for a child with developmental disabilities, who is more likely to continue living in the family home throughout adulthood than other adult children. Federal and state initiatives are addressing issues of family support through both the developmental disabilities and aging service systems.     

Unique pathways to resilience across cultures

An international mixed methods study of resilience of 14 sites in eleven countries identified seven tensions that youth resolve in culturally specific ways. Resolution of these tensions is foundational to experiences of resilience. This paper reports on the qualitative findings from interviews with 89 youth. Results support a culturally embedded understanding of positive youth development that better accounts for young people's resilience in western and non-western countries. Specifically, the seven tensions identified include: access to material resources, relationships, identity, cohesion, power and control, social justice, and cultural adherence. Findings show that no one pattern in the resolution of these tensions predicts resilience better than another. A case study of a Palestinian boy demonstrates the intersection of the seven tensions and the uniqueness of their resolution. The implications of this work for interventions is discussed.     

On the Distinction between the Abstract Tacts Art and Craft: A Concept Analysis

The Psychological Record - We may marvel at both the creativity and quality of work in a piece of pottery or a sculpture, or of a well-played basketball game or well-acted play. Although each may...     

Can God or the Market Set People Free?

Both Protestant theologians and “preference” economists believe that freedom is necessary for moral action, but such theologians and economists have seemingly irreconcilable accounts of freedom and, thus also, morality. Instead of learning from each other, they typically ignore each other or claim that one field reigns supreme over the other. This essay digs into the theological and economic traditions of each side to find points of similarity between them. It engages Adam Smith and Ernst Troeltsch to develop a view of “ethical freedom” that pulls together the “libertarian freedom” emphasized by preference economics and the “egalitarian freedom” emphasized in much Protestant theology. The three‐part view of freedom draws the disciplines together and opens possibilities for a more robust theory of moral action.     

Connectedness and its consequences: a study of relationships with the natural environment

Recent interest in people's engagement with the natural environment has resulted in a series of empirical measures and important research findings. Two studies reported here complement this literature in producing a concise measure of connectedness to the natural environment and assessing its independent predictive impact within the structure of the theory of planned behavior. In Study 1 (n = 71), new measures of connectedness and caring were constructed; in Study 2 (n = 163), the connectedness measure was shown to be an independent predictor of people's intentions to reduce personal energy consumption levels. It is suggested that the measure of connectedness may capture some identity‐related and/or affective experience that impacts (independently of more cognitive predictors) upon people's motivation in this domain.     

Wild robins (Petroica longipes) respond to human gaze

Gaze following and awareness of attentional cues are hallmarks of human and non-human social intelligence. Here, we show that the North Island robin (Petroica longipes), a food-hoarding songbird endemic to New Zealand, responds to human eyes. Robins were presented with six different conditions, in which two human experimenters altered the orientation or visibility of their body, head or eyes in relation to mealworm prey. One experimenter had visual access to the prey, and the second experimenter did not. Robins were then given the opportunity to ‘steal’ one of two mealworms presented by each experimenter. Robins responded by preferentially choosing the mealworm in front of the experimenter who could not see, in all conditions but one. Robins failed to discriminate between experimenters who were facing the mealworm and those who had their head turned 90° to the side. This may suggest that robins do not make decisions using the same eye visibility cues that primates and corvids evince, whether for ecological, experiential or evolutionary reasons.     

“Decisions,decisions, decisions”: transfer and specificity of decision-making skill between sports

The concept of transfer of learning holds that previous practice or experience in one task or domain will enable successful performance in another related task or domain. In contrast, specificity of learning holds that previous practice or experience in one task or domain does not transfer to other related tasks or domains. The aim of the current study is to examine whether decision-making skill transfers between sports that share similar elements, or whether it is specific to a sport. Participants (n = 205) completed a video-based temporal occlusion decision-making test in which they were required to decide on which action to execute across a series of 4 versus 4 soccer game situations. A sport engagement questionnaire was used to identify 106 soccer players, 43 other invasion sport players and 58 other sport players. Positive transfer of decision-making skill occurred between soccer and other invasion sports, which are related and have similar elements, but not from volleyball, supporting the concept of transfer of learning.