Behavioral profile of adults with Prader-Willi syndrome : correlations with individual and environmental variables

Background: Maladaptive behavior has been reported as a phenotypical feature in Prader–Willi syndrome (PWS). It severely limits social adaptation and the quality of life of children and adults with the syndrome. Different factors have been linked with the intensity and form of these behavioral disturbances but there is no consensus about the cause. Consequently, there is still controversy regarding management strategies and there is a need for new data. Methods: The behavior of 100 adults with PWS attending a dedicated center was assessed using the Developmental Behavior Checklist for Adults (DBC-A) and the PWS-specific Hyperphagia Questionnaire. The DBC-A was completed separately by trained caregivers at the center and relatives or caregivers in a natural setting. Genotype, gender, age, degree of obesity and cognitive impairment were analyzed as variables with a hypothetical influence on behavioral features. Results: Patients showed a relatively high rate of behavioral disturbances other than hyperphagia. Disruptive and social relating were the highest scoring DBC-A subscales whereas anxiety/antisocial and self-absorbed were the lowest. When hospital caregiver and natural caregiver scores were compared, scores for the latter were higher for all subscales except for disruptive and anxiety/antisocial. These effects of institutional management were underlined. In the DBC-A, 22 items have descriptive indications of PWS behavior and were used for further comparisons and correlation analysis. In contrast to previous reports, rates of disturbed behavior were lower in patients with a deletion genotype. However, the behavioral profile was similar for both genotypes. No differences were found in any measurement when comparing type I and type II deletions. The other analyzed variables showed little relevance. Conclusions: Significant rates of behavioral disorders were highlighted and their typology described in a large cohort of adults with PWS. The deletion genotype was related to a lower severity of symptoms. Some major behavioral problems, such as hyperphagia, may be well controlled if living circumstances are adapted to the specific requirements of individuals with PWS.

Proposal and validation of methodologies for the categorisation of continuous variables in the development of prediction models

158 p.

Physiological modules for generating discrete and rhythmic movements: Component analysis of EMG signals

A central question in Neuroscience is that of how the nervous system generates the spatiotemporal commands needed to realize complex gestures, such as handwriting. A key postulate is that the central nervous system (CNS) builds up complex movements from a set of simpler motor primitives or control modules. In this study we examined the control modules underlying the generation of muscle activations when performing different types of movement: discrete, point-to-point movements in eight different directions and continuous figure-eight movements in both the normal, upright orientation and rotated 90 degrees. To test for the effects of biomechanical constraints, movements were performed in the frontal-parallel or sagittal planes, corresponding to two different nominal flexion/abduction postures of the shoulder. In all cases we measured limb kinematics and surface electromyographic activity (EMB) signals for seven different muscles acting around the shoulder. We first performed principal component analysis (PCA) of the EMG signals on a movement-by-movement basis. We found a surprisingly consistent pattern of muscle groupings across movement types and movement planes, although we could detect systematic differences between the PCs derived from movements performed in each sholder posture and between the principal components associated with the different orientations of the figure. Unexpectedly we found no systematic differences between the figute eights and the point-to-point movements. The first three principal components could be associated with a general co-contraction of all seven muscles plus two patterns of reciprocal activatoin. From these results, we surmise that both "discrete-rhythmic movements" such as the figure eight, and discrete point-to-point movement may be constructed from three different fundamental modules, one regulating the impedance of the limb over the time span of the movement and two others operating to generate movement, one aligned with the vertical and the other aligned with the horizontal.

Influence of nutrient intake on antioxidant capacity, muscle damage and white blood cell count in female soccer players

11 p.

Daily scale wintertime sea surface temperature and IPC-Navidad variability in the southern Bay of Biscay from 1981 to 2010

The combination of remotely sensed gappy Sea surface temperature (SST) images with the missing data filling DINEOF (data interpolating empirical orthogonal functions) technique, followed by a principal component analysis of the reconstructed data, has been used to identify the time evolution and the daily scale variability of the wintertime surface signal of the Iberian Poleward Current (IPC), or Navidad, during the 1981-2010 period. An exhaustive comparison with the existing bibliography, and the vertical temperature and salinity profiles related to its extremes over the Bay of Biscay area, show that the obtained time series accurately reflect the IPC-Navidad variability. Once a time series for the evolution of the SST signal of the current over the last decades is well established, this time series is used to propose a physical mechanism in relation to the variability of the IPC-Navidad, involving both atmospheric and oceanic variables. According to the proposed mechanism, an atmospheric circulation anomaly observed in both the 500 hPa and the surface levels generates atmospheric surface level pressure, wind-stress and heat-flux anomalies. In turn, those surface level atmospheric anomalies induce mutually coherent SST and sea level anomalies over the North Atlantic area, and locally, in the Bay of Biscay area. These anomalies, both locally over the Bay of Biscay area and over the North Atlantic, are in agreement with several mechanisms that have separately been related to the variability of the IPC-Navidad, i.e. the south-westerly winds, the joint effect of baroclinicity and relief (JEBAR) effect, the topographic beta effect and a weakened North Atlantic gyre.

Divergences in the Long Run Trends in the Price of Energy and of Energy Services

32 p.

Increased antiparkinson efficacy of the combined administration of VEGF- and GDNF-loaded nanospheres in a partial lesion model of Parkinson's disease

Current research efforts are focused on the application of growth factors, such as glial cell line-derived neurotrophic factor (GDNF) and vascular endothelial growth factor (VEGF), as neuroregenerative approaches that will prevent the neurodegenerative process in Parkinson's disease. Continuing a previous work published by our research group, and with the aim to overcome different limitations related to growth factor administration, VEGF and GDNF were encapsulated in poly(lactic-co-glycolic acid) nanospheres (NS). This strategy facilitates the combined administration of the VEGF and GDNF into the brain of 6-hydroxydopamine (6-OHDA) partially lesioned rats, resulting in a continuous and simultaneous drug release. The NS particle size was about 200 nm and the simultaneous addition of VEGF NS and GDNF NS resulted in significant protection of the PC-12 cell line against 6-OHDA in vitro. Once the poly(lactic-co-glycolic acid) NS were implanted into the striatum of 6-OHDA partially lesioned rats, the amphetamine rotation behavior test was carried out over 10 weeks, in order to check for in vivo efficacy. The results showed that VEGF NS and GDNF NS significantly decreased the number of amphetamine-induced rotations at the end of the study. In addition, tyrosine hydroxylase immunohistochemical analysis in the striatum and the external substantia nigra confirmed a significant enhancement of neurons in the VEGF NS and GDNF NS treatment group. The synergistic effect of VEGF NS and GDNF NS allows for a reduction of the dose by half, and may be a valuable neurogenerative/neuroreparative approach for treating Parkinson's disease.

Socioeconomic status, parental support, motivation and self-confidence in youth competitive sport

World Conference on Psychology and Sociology 2012

Number of Players and Relative Pitch Area per Player: Comparing Their Influence on Heart Rate and Physical Demands in Under-12 and Under-13 Football Players

The aim of the present study is to analyse the influence of different large-sided games (LSGs) on the physical and physiological variables in under-12s (U12) and -13s (U13) soccer players. The effects of the combination of different number of players per team, 7, 9, and 11 (P7, P9, and P11, respectively) with three relative pitch areas, 100, 200, and 300 m(2) (A100, A200, and A300, respectively), were analysed in this study. The variables analysed were: 1) global indicator such as total distance (TD); work:rest ratio (W:R); player-load (PL) and maximal speed (V-max); 2) heart rate (HR) mean and time spent in different intensity zones of HR (<75%, 75-84%, 84-90% and >90%), and; 3) five absolute (<8, 8-13, 13-16 and >16 Km h(-1)) and three relative speed categories (<40%, 40-60% and >60% V-max). The results support the theory that a change in format (player number and pitch dimensions) affects no similarly in the two players categories. Although it can seem that U13 players are more demanded in this kind of LSG, when the work load is assessed from a relative point of view, great pitch dimensions and/or high number of player per team are involved in the training task to the U12 players. The results of this study could alert to the coaches to avoid some types of LSGs for the U12 players such as:P11 played in A100, A200 or A300, P9 played in A200 or A300 and P7 played in A300 due to that U13>U12 in several physical and physiological variables (W:R, time spent in 84-90% HRmax, distance in 8-13 and 13-16 Km h(-1) and time spent in 40-60% V-max). These results may help youth soccer coaches to plan the progressive introduction of LSGs so that task demands are adapted to the physiological and physical development of participants.