Theory of Mind impairment in childhood narcolepsy type 1: a case-control study.

Narcolepsy type 1 is a central disorder of hypersomnolence characterized by excessive daytime sleepiness, cataplexy and other rapid eye movement sleep-related manifestations. Neurophysiological studies suggest that narcolepsy type 1 patients may experience impairment in emotional processing due to structural and functional changes in limbic structures and associated areas. However, the only study exploring narcolepsy behavioural responses found no impairment in the ability to recognize emotions, possibly due to compensatory mechanisms. The present study was designed to fill this gap in the literature by investigating the behavioural impairment related to emotional processing focusing on an advanced socio-cognitive skill, namely Theory of Mind, in paediatric narcolepsy type 1 patients. Twenty-two narcolepsy type 1 children and adolescents (six female; age range: 8.0-13.5) and 22 healthy controls matched for age and sex (six female; age range: 8.9-13.0) underwent a neuropsychological evaluation to assess socio-economic status, verbal abilities, working memory, social anxiety and Theory of Mind via a verbal task (i.e. Strange Stories task) and a visual task (i.e. Silent Films). Narcolepsy type 1 patients were also evaluated for disease severity. Patients exhibited impairment in Theory of Mind skills, as assessed both through both verbal (controls median = 8; patients median = 5; P = 0.009) and visual tasks (controls median = 8; patients median = 6; P = 0.003), compared to healthy controls. Correlation analyses showed that verbal and visual Theory of Mind was negatively related to narcolepsy severity (ρ = -0.45, P = 0.035 and ρ = -0.52, P = 0.012), and daytime sleepiness (ρ = -0.48, P = 0.025 and ρ = -0.45, P = 0.038). Our study shows a selective impairment in the Theory of Mind domain in children and adolescents with narcolepsy type 1. In addition, our results highlight a link between symptom severity and Theory of Mind, suggesting that lower Theory of Mind levels are associated with higher symptom severity. Further, longitudinal studies are needed to disentangle the direction of this relation and to disambiguate if narcolepsy severity impaired children's Theory of Mind or if Theory of Mind skills modulate the severity of narcolepsy symptoms by providing a greater ability to avoid cataplexy.

Space For Children: improving mastery and reducing negative emotions about COVID-19 in youth via mobile interactive storytelling.

BACKGROUND: The COVID-19 pandemic has been a complex event for children and adolescents, significantly impacting their daily activities. In this scenario, our study aims at verifying if "Space for Children," an ad-hoc developed game based on interactive storytelling about the pandemic, can increase mastery and reduces negative emotions about COVID-19. METHODS: The app Space for Children has been sponsored and spread online by email and social media platforms for a limited time period (from May 2021 to January 2022). Before and after the interactive storytelling, participants answered two questionnaires containing a couple of critical questions regarding their emotional state and their perceived mastery about COVID-19. RESULTS: Two hundred thirty-six participants (M=116; F=120) between 7 and 12 years old completed the Space for Children interactive experience. Our results show a significant effect of the game experience on emotional state and perceived mastery regarding COVID-19. Indeed, before the interactive storytelling unpleasant emotional states prevail (reported by 77% of participants) while after the game a clear prevalence of pleasant emotions emerges (reported by 68% of participants). Regarding perceived mastery, results show that self-reported very expert participants passed from 49% to 88%. All the results hold irrespective of age group and sex. CONCLUSIONS: This study presents preliminary findings that highlight the potential benefits of utilizing new technologies based on Interactive Storytelling for Children to effectively convey age-appropriate information about complex real-life events, such as the COVID-19 pandemic, while also mitigating associated negative emotional responses.

Narcolepsy and emotions: Is there a place for a theory of mind approach?

Narcolepsy type 1 is a central disorder of hypersomnolence characterized by excessive daytime sleepiness, rapid eye movement sleep-related manifestations, and cataplexy. In the current literature there is general agreement regarding neural correlates of Narcolepsy type 1 that appear to be related to anatomical and functional abnormalities in the hypothalamic region. In the last two decades, researchers shed light on the neurological bases of cataplexy by focusing on the neurobiological correlates of emotions. Although the results of these studies differ, they all point to an impairment in the amygdala and hypothalamus functions that are known to be involved in emotional processing, suggesting an impairment in this domain in narcoleptic patients. Indeed, despite heterogeneous results, several studies showed that narcoleptic patients differed from healthy controls in processing emotional stimuli. From a behavioral point of view, these findings suggest that alterations in emotional processing may be driven, at least in part, by compensatory strategies to avoid or reduce the frequency of cataplexy attacks. Surprisingly, the only study exploring in NT1 the behavioural performances in emotional facial recognition found no differences between NT1 adults and controls. We hypothesize that narcoleptic patients may present an alteration in a more complex socio-cognitive ability that is related to emotional processing, namely Theory of Mind. This review aims to investigate the literature supporting this hypothesis and to propose possible future developments on this topic.

The pandemic seen through the eyes of the youngest people: evaluating psychological impact of the early COVID-19 related confinement on children and adolescents through the analysis of drawings and of an e-survey on their parents.

BACKGROUND: COVID-19 is having a significant impact on long term children' and adolescents' psychological health. We aimed to evaluate the direct early psychological and behavioural signs related to the COVID-19 pandemic outbreak and related confinement on children and adolescents. METHODS: Children and adolescents' drawings were collected for a limited time window (16th March-10th April 2020) and analyzed. Their parents were asked in the following month to answer a qualitative e-survey on somatic complaints and behavioral changes of the participating children/adolescents. RESULTS: Ninety-eight drawings by children/adolescents (mean age 7.01±2.83 years) were analysed. Analyses of the 98 drawings reported signs of trauma in all (of them, 60.2% with moderate-to high levels). Children aged 3-5 years were more impacted, followed by preadolescents/adolescents aged 11-17 years. Parents reported somatic complaints in the 71.1% of their children/adolescents: the most frequent were increased appetite (35.6%), abdominal pain (20.0%) and headache (20.0%). Behavioral changes were observed in 77.8% of subjects: increased appetite (35.6%), abdominal pain (20.0%) and headache (20.0%) were more represented. CONCLUSIONS: Early psychological distress related to COVID-19 pandemic was observed both in children and in adolescents by the analysis of drawings and confirmed by their parents. Implementation of mental health-care services for preventing future psychopathological problems is mandatory.

Depressive Anxiety Symptoms in Hospitalized Children with Chronic Illness during the First Italian COVID-19 Lockdown.

COVID-19 is continuing to spread around the world, having a direct impact on people's daily lives and health. Although the knowledge of the impact of the COVID-19 pandemic on mental health in the general population is now well established, there is less information on its effect on specific and vulnerable populations, such as children with chronic illness (CI). We conducted a multi-centered cross-sectional study among pediatric patients in six public children's hospitals in Italy during the first lockdown, with the aim of assessing the proportion of children with CI presenting anxiety and depressive symptoms, and the clinical and demographic characteristics affecting such symptomatology. We included children with at least one chronic condition, with no cognitive delay, aged between 11 and 18 years. Brief standardized questionnaires were administered during medical scheduled visits to screen anxiety and depressive symptoms. We found a very high proportion of children showing mild to severe depressive and anxiety symptomatology (approximately 68% and 63%, respectively). Our results highlight the need of ensuring tailored psychological interventions to protect children with CI from the effect of the pandemic (and related restrictive measures such as quarantine and social distancing), with the final aim of promoting mental health and psychological well-being in this vulnerable population.

Impact of the COVID-19 Outbreak on the Behavior of Families in Italy: A Focus on Children and Adolescents.

The COVID-19 pandemic has changed individuals' lifestyles to a great extent, particularly in Italy. Although many concerns about it have been highlighted, its impact on children and adolescents has scarcely been examined. The purpose of this study was to explore behavioral consequences and coping strategies related to the pandemic among families in Italy, by focusing on developmental ages from the caregivers' perspective, 3 weeks into quarantine. An exploratory cross-sectional online survey was conducted over 14 days. Google Forms was employed to conduct the survey. Demographic variables and pre-existing Psychological Weaknesses (PsW) were asked. Adults' sleep difficulties (SleepScore) and coping strategies during quarantine were assessed. Behavioral changes related to quarantine of both subjects completing the form (COVIDStress) and their children (when present) were questioned. Of the 6,871 respondents, we selected 6,800 valid questionnaires; 3,245 declared children aged under 18 years of age (caregivers). PsWs were recognizable in 64.9% among non-caregivers and in 61.5% of caregivers, with a mean PsW score of 1.42 ± 1.26 and 1.30 ± 1.25 over 3 points, respectively. The 95.5% of the non-caregivers and the 96.5% of caregivers presented behavioral changes with a mean COVIDStress of 3.85 ± 1.82 and 4.09 ± 1.79 over 8, respectively (p<0.001). Sleep difficulties were present in the 61.6% of the non-caregivers and in the 64.4% of the caregivers (p < 0.001), who showed higher SleepScores (2.41 ± 1.26 against 2.57 ± 1.38 points over 6, p < 0.001). COVIDStress (and SleepScore) strongly correlated with PsW (p < 0.001). Caregivers observed behavioral changes in their children in the 64.3% of the <6 years old and in 72.5% of 6-18 years old. Caregivers' discomfort related to quarantine (COVIDStress, SleepScore) was strongly associated to behavioral changes in both age groups of <6 and 6-18 (p < 0.001). Presence of caregivers' coping strategies was less associated to behavioral changes in the <6 sample (p = 0.001) but not in the 6-18 (p = 0.06). The COVID-19 pandemic has adversely impacted families in Italy with regard to behavioral changes, especially in high-risk categories with PsWs and caregivers, especially the ones with children aged <6 years. While coping strategies functioned as protective factors, a wide array of stress symptoms had implications for children's and adolescents' behaviors. It is recommended that public children welfare strategies be implemented, especially for higher-psychosocial-risk categories.

Consensus based recommendations for diagnosis and medical management of Poland syndrome (sequence).

BACKGROUND: Poland syndrome (OMIM: 173800) is a disorder in which affected individuals are born with missing or underdeveloped muscles on one side of the body, resulting in abnormalities that can affect the chest, breast, shoulder, arm, and hand. The extent and severity of the abnormalities vary among affected individuals. MAIN BODY: The aim of this work is to provide recommendations for the diagnosis and management of people affected by Poland syndrome based on evidence from literature and experience of health professionals from different medical backgrounds who have followed for several years affected subjects. The literature search was performed in the second half of 2019. Original papers, meta-analyses, reviews, books and guidelines were reviewed and final recommendations were reached by consensus. CONCLUSION: Being Poland syndrome a rare syndrome most recommendations here presented are good clinical practice based on the consensus of the participant experts.

Changes in the quality of life of people with thalassemia major between 2001 and 2009.

BACKGROUND: The prolonged survival of patients with thalassemia major as a result of the novel therapeutic strategies introduced in the last decade makes patient quality of life an important issue. This study investigated the changes occurring in overall quality of life in patients with thalassemia in the last decade. METHODS: This was a population-based cross-sectional survey of quality of life in the entire population with thalassemia major resident in the Liguria region of Italy from 2001 to 2009. The self-administered Short Form-36 (SF-36) questionnaire was used to measure quality of life in patients with thalassemia. RESULTS: Forty-nine and 52 eligible patients were assessed in 2001 and 2009, respectively. A total of 43 patients were assessed in both 2001 and 2009. Almost 40% of these 43 patients received deferasirox in 2009, a drug which was not available in 2001. The distribution of ferritin levels was lower in 2009 (median 730) as compared with 2001 (median 1107). Analysis of the raw differences between the two years did not show a significant difference. An improvement was observed in most SF-36 scales in 2009 as compared with 2001, particularly in the Mental Health scale (mean difference in Z score +4.0; 95% confidence interval 0.4-7.5; P = 0.030) and in the Mental Component Summary scale (mean difference in Z score +3.2; 95% confidence interval 0.2-6.2; P = 0.039). CONCLUSION: The challenge associated with new therapies and improvement in mental quality of life dimensions indicates that implementation of effective interventions for screening and evaluation of quality of life is now urgent.