Mutations in the SPAST gene causing hereditary spastic paraplegia arerelated to global topological alterations in brain functional networks

Aim: Our aim was to describe the rearrangements of the brain activity related to genetic mutations in the SPAST gene. Methods: Ten SPG4 patients and ten controls underwent a 5 min resting state magnetoencephalography recording and neurological examination. A beamformer algorithm reconstructed the activity of 90 brain areas. The phase lag index was used to estimate synchrony between brain areas. The minimum spanning tree was used to estimate topological metrics such as the leaf fraction (a measure of network integration) and the degree divergence (a measure of the resilience of the network against pathological events). The betweenness centrality (a measure to estimate the centrality of the brain areas) was used to estimate the centrality of each brain area. Results: Our results showed topological rearrangements in the beta band. Specifically, the degree divergence was lower in patients as compared to controls and this parameter related to clinical disability. No differences appeared in leaf fraction nor in betweenness centrality. Conclusion: Mutations in the SPAST gene are related to a reorganization of the brain topology

Critical Success Factors in Strategic Brand Management in Luxury Fashion Markets: The Case of Isaia

This chapter examines the critical role of branding in sustaining the competitive advantage of firms in the luxury fashion markets. This can include brand communication, the flagship store, channel relationships, the customization and the event marketing. The chapter sets out an analytical framework that can be used to identify the main critical success factors in the fashion luxury market. Adopting the case study method, we validate this framework by studying a pilot firm, Isaia, which is a traditional Italian firm operating in the fashion luxury industry (clothing for men). Isaia is an interesting case study of the branding strategies of a niche fashion firm in the face of global competition. Through the original framework, we have identified the company’s main critical success factors that—in the exploratory stage of our research—support the work purposes. In particular, the main findings from the case study demonstrate that Isaia’s competitive advantage is found on customized products (luxury traditions), events (dinner parties, in‐store entertainment, etc.) and retail strategy (flagship showrooms in Milan and Moscow)

ADDICTIONS SUBSTANCE FREE DURING LIFESPAN

The addictions substance free is an umbrella definition comprises internet addiction, sexual addiction, gambling pathological, workholism, videogames and computer addiction. Actually, the technological addictions is frequent in young adolescents. The term Digital Natives indicates the children born in an information system of learning and communication different from that of the generations previous. This temporal range was strongly characterized by growing presence of technological communication toolsin daily life. The effects of hyper-exposition to technological tools tend to create a relational virtuality without a body is born,therefore, already within the family ties and during adolescence he moved to the digital socialization network. The technological object it interacts between the adolescent and the world of peers and adults, becoming the facilitator object that as the psychotropic substance, it conveys new modes of communicatio

Behavioral aspects in children's brothers affected by Autism Spectrum Disorders

Introduction: Autistic Spectrum Disorder (ASD) is a permanent and complex disability arising within the first three years of life characterized by a socio-communicative disorder and by fixed interests and repetitive behaviors. The present pilot study aims to evaluate behavioral aspects in a small population of siblings of ASD children. Material and methods: Population: 5 school-aged children (2 males, 3 females) (mean age 9.235 ± 2.041) were enrolled, as siblings of ASD children, and for comparison, 12 healthy (7 males, 5 females) children (average age 9,528 ± 3,351). All subjects underwent evaluation of the behavioral with Child Behavior Checklist (CBCL) scale. Results: The two groups were statistically comparable by age (p = 0.86) and gender distribution (p = 0.87). From the behavioral point of view evaluated with the CBCL scale, siblings of ASD have a higher degree of overall problem (Total problems) compared to control children (p=0.003), in addition they have significantly higher scores in the subscales of behavior examined (Anxious/Depressed, Withdrawn, Somatic Complaints, Social, Thought, Attention, Delinquent, Aggressive) as well as a greater share of disturbances both internalizing (p=0.004) and externalizing (p = 0.007) (Table 1). Conclusions: The present preliminary data confirm the need for a global management of the entire family structure for the correct management of Autistic Disorders

When Care Becomes Abuse: A Forensic–Medical Perspective on Munchausen Syndrome by Proxy

Munchausen Syndrome by Proxy (MSBP) is recognized as a form of child abuse in which a caregiver induces or fabricates illnesses in their child to gain medical and social attention. MSBP represents one of the most complex and insidious forms of child abuse, characterized by an ambiguous clinical presentation that poses significant challenges for physicians, psychiatrists, and social workers. However, this condition raises critical questions regarding its diagnosis, management, and forensic implications. Traditionally, MSBP has been framed as an individual pathological manifestation of the mother, overlooking the role of the healthcare and legal systems in its identification and management. In this article, we propose a critical reflection on MSBP, emphasizing how the issue is not merely a “parental failure” but rather a systemic failure of healthcare, social, and judicial institutions in recognizing, preventing, and effectively managing such cases

Static Behavior of a 3D-Printed Short Carbon Fiber Polyamide: Influence of the Meso-Structure and Water Content

The knowledge of the mechanical behavior of a 3D-printed material is fundamental for the 3D printing outbreaking technology to be considered for a range of applications. In this framework, the significance, reliability, and accuracy of the information obtained by testing material coupons assumes a pivotal role. The present work focuses on an evaluation of the static mechanical properties and failure modes of a 3D-printed short carbon fiber-reinforced polyamide in relation to the specimen’s unique meso-structural morphology and water content. Within the manufacturing limitations of a commercially available printer, specimens of dedicated combinations of geometry and printing patterns were specifically conceived and tested. The specimens’ meso-structure morphologies were investigated by micro-computed tomography. The material failure mechanisms were inferred from an analysis of the specimens’ fracture surfaces and failure morphologies. The outcomes of the present analysis indicate that each test specimen retained proper mechanical properties, thereby suggesting that they should be accurately designed to deliver representative information of the underlying material beads or of their deposition layout. Suggestions on the adoption of preferred test specimens for evaluating specific material properties were proposed