A Case of Partial Biotinidase Deficiency Associated With Autism

We report the case of a child with partial biotinidase deficiency and autistic developmental disorder. We arrived at the diagnosis of biotinidase deficiency when the child was almost 4 years of age. Consequently, he began cofactor biotin treatment (10 mg daily) which did not resolve his autistic behavior. His younger brother was affected by partial biotinidase deficiency diagnosed at birth through our neonatal screening program. He was precociously treated with cofactor biotin therapy (10 mg daily) and did not show any behavioral abnormality or developmental delay. Since the brain is quite vulnerable to biotin deficiency, delayed biotin therapy could result in neurological damage. Our patient is the first case of partial biotinidase deficiency associated with autism. We hypothesize that the low biotinidase activity could have caused biotin deficiency in his brain and cerebrospinal fluids and consequently serious neurological problems, such as stereotyped and autistic behaviors, which were irreversible in spite of biotin supplementation.     

Psychometric analysis of the AF5 multidimensional scale of self-concept in a sample of adolescents and adults in Catalonia

The aim of this study is to carry out a psychometric study of the AF5 scale in a sample of 4.825 Catalan subjects from 11 to 63 years-old. They are students from secondary compulsory education (ESO), from high school, middle-level vocational training (CFGM) and from the university. Using a principal component analysis (PCA) the theoretical validity of the components is established and the reliability of the instrument is also analyzed. Differential analyses are performed by gender and normative group using a 2 x 6 factorial design. The normative group variable includes the different levels classified into 6 sub-groups: university, post-compulsory secondary education (high school and CFGM), 4th of ESO, 3rd of ESO, 2nd of ESO and 1st of ESO. The results indicate that the reliability of the Catalan version of the scale is similar to the original scale. The factorial structure also fits with the original model established beforehand. Significant differences by normative group in the four components of self-concept explored (social, family, academic/occupational and physical) are observed. By gender, significant differences appear in the component of physical self-concept, academic and social but not in the family component.     

Assessing human reorientation ability inside virtual reality environments: the effects of retention interval and landmark characteristics

The purpose of the present study was to assess the navigational behaviour of adult humans following a disorientation procedure that perturbed their egocentric frame of reference. The assessment was carried out in a virtual reality (VR) environment by manipulating the disorientation procedure, the retention interval, the relative positions of target and landmark. The results of experiment I demonstrated that adding a physical rotation to a virtual disorientation procedure did not yield an additional decrease in searching performance. The results of experiment II showed that shortening the delay between study and test phase decreased the errors more markedly for geometric than landmark ones. An orientation specificity effect due to the manipulation of the relative position between target and landmark was discussed across the experiments. In conclusion, VR seemed to be a valuable method for studying human reorientation. Moreover, the virtual experimental setting involved here promoted knowledge of the relationship between working memory and spatial reorientation paradigm.     

A case of partial biotinidase deficiency associated with autism.

We report the case of a child with partial biotinidase deficiency and autistic developmental disorder. We arrived at the diagnosis of biotinidase deficiency when the child was almost 4 years of age. Consequently, he began cofactor biotin treatment (10 mg daily) which did not resolve his autistic behavior. His younger brother was affected by partial biotinidase deficiency diagnosed at birth through our neonatal screening program. He was precociously treated with cofactor biotin therapy (10 mg daily) and did not show any behavioral abnormality or developmental delay. Since the brain is quite vulnerable to biotin deficiency, delayed biotin therapy could result in neurological damage. Our patient is the first case of partial biotinidase deficiency associated with autism. We hypothesize that the low biotinidase activity could have caused biotin deficiency in his brain and cerebrospinal fluids and consequently serious neurological problems, such as stereotyped and autistic behaviors, which were irreversible in spite of biotin supplementation.     

Investigating how internet gaming disorder and bodily dissociation experiences vary by game genres

Cognitive Processing - This study aims to investigate whether Internet gaming disorder (IGD) and bodily dissociation vary depending on the most frequently used game genre. Seven hundred and fifteen...     

The child's sense of existence and the fatal attraction of identification

This paper gives special emphasis to the widening and modification of the sense of existence in relation to the unconscious processes of identification. In our ‘post‐modern’ society there are some parents who, while taking care of their children, use subtle projective identification against them, so as to produce in them a sort of pathological introjective identification, which denies their Self. Ferenczi's thought, which deals with the sense of ‘not‐existing’ in some children, helps us to go into depth in understanding their difficulties and pathologies.     

Learning the Ropes: The Protective Role of Mentoring in Correctional Police Officers’ Socialization Process

Formal mentoring is an individualized and contextualized socialization tactic to enhance newcomers’ learning—acknowledged as essential in the early career stage—that can be of particular value when entering a fairly unpredictable and stressful workplace. This research aims to understand the moderating role of formal mentoring in the relationship between organizational socialization and 2 adjustment indicators, a positive 1 (commitment) and a negative 1 (turnover intention). A questionnaire was administered to 117 correctional police officer newcomers, as prisons are especially critical work contexts for newcomers. The results show a direct effect from both socialization and mentoring on commitment and turnover, and an interaction between socialization and mentoring on turnover, although not on commitment. When the socialization process progresses steadily, both socialization and mentoring contribute to good adjustment, but when traditional tactics go wrong, a different learning source (formal mentoring) exerts a protective function, limiting newcomers’ intention to quit. These findings give support to the usefulness of mentoring in a law enforcement context and provide some insight into defining formal mentoring programs.     

Socio-cultural variation in attitudes to adolescents’ decision-making

Little is known about cultural variation in perceptions about the age when youngsters should be regarded as competent to make decisions. This is of particular importance as research has shown the significant effect of parental influence on their children’s decision-making. A public survey of 400 adults from diverse socio-cultural backgrounds in the UK and Spain was carried out. Their attitudes regarding adolescents’ ability with regard to common areas of decision-making were assessed using case vignettes including discontinuing family traditions and religious practice. It was found that White British adults agreed for younger adolescents to make decisions about themselves compared with the other ethnic groups. High religious practice was associated with later age to make decisions. The paper argues that there is socio-cultural variation in adults’ attitudes regarding the age when youngsters should make decisions about themselves and for the need of professionals to take this into account.