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.

Virtual Sensors for On-line Wheel Wear and Part Roughness Measurement in the Grinding Process

Grinding is an advanced machining process for the manufacturing of valuable complex and accurate parts for high added value sectors such as aerospace, wind generation, etc. Due to the extremely severe conditions inside grinding machines, critical process variables such as part surface finish or grinding wheel wear cannot be easily and cheaply measured on-line. In this paper a virtual sensor for on-line monitoring of those variables is presented. The sensor is based on the modelling ability of Artificial Neural Networks (ANNs) for stochastic and non-linear processes such as grinding; the selected architecture is the Layer-Recurrent neural network. The sensor makes use of the relation between the variables to be measured and power consumption in the wheel spindle, which can be easily measured. A sensor calibration methodology is presented, and the levels of error that can be expected are discussed. Validation of the new sensor is carried out by comparing the sensor's results with actual measurements carried out in an industrial grinding machine. Results show excellent estimation performance for both wheel wear and surface roughness. In the case of wheel wear, the absolute error is within the range of microns (average value 32 mu m). In the case of surface finish, the absolute error is well below R-a 1 mu m (average value 0.32 mu m). The present approach can be easily generalized to other grinding operations.