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Background: The aim of the study was to explore the subjective perception of their own sleep and daytime habits in (pre-)adolescents with chronic diseases., Methods: Self-administered questionnaires exploring daytime and nighttime habits, health behavior, daytime sleepiness, depression and anxiety were fulfilled by the (pre-)adolescents., Results: Hundred sixty-one patients with a chronic disease, aged 14.3±2.6 years old, participated to the study. Mean total time in bed was 8h52±1h09 (range 5h00-11h30) on school days (TIBS) and 9h59±1h28 (range 6h00-14h00) on non-school days (TIBN), with 11 (7%) adolescents reporting sleeping ≤7 hours during school days. The mean sleep time difference between TIBS and TIBN was 67±95 minutes (range: 210-330 min), with 33 patients (20%) having a sleep debt>2 h, and 38% reporting sleep initiating problems. Patients with cystic fibrosis had the lowest mean TIBS, the highest percentage (37%) of patients with sleep debt >2 h. Obese patients were the sleepiest (33%) with 8% having sleep debt. Anxiety and severe depression were observed in 22% and 20% of the patients, respectively, and correlated with fatigue at wake up and daytime sleepiness., Conclusions: In these (pre-)adolescents with a chronic disease, 20% had sleep debt but sleep duration was reasonable with acceptable respect of sleep hygiene rules.

Neuronal ceroid lipofuscinosis type 2 (CLN2) disease is a rare, lysosomal storage disorder that causes pediatric onset neurodegenerative disease. It is characterized by mutations in the TPP1 gene. Symptoms begin between 2 and 4 years of age with loss of previously acquired motor, cognitive, and language abilities. Cerliponase alfa, a recombinant human TPP1 enzyme, is the only approved therapy. We report the first presymptomatic cerliponase alfa intraventricular treatment in a familial case of CLN2 related to a classical TPP1 variant. Sister 1 presented with motor, cognitive, and language decline and progressive myoclonic epilepsy since the age of 3 years, evolved with severe diffuse encephalopathy, received no specific treatment, and died at 11 years. Sister 2 had a CLN2 presymptomatic diagnosis and has been treated with cerliponase since she was 12 months old. She is now 6 years 8 months and has no CLN2 symptom except one generalized seizure 1 year ago. No serious adverse event has occurred. Repeated Wechsler Preschool and Primary Scale of Intelligence, Fourth Edition standardized index scores are heterogeneous in the extremely low to low average ranges. Mean length of utterances, a global index of sentence complexity, showed a delay, but a gradual improvement. The reported case enhances the major contribution of presymptomatic diagnosis and significant middle-term treatment benefit for patients with CLN2., (© 2024 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Current psychopathology models have evolved toward dimensional models, in which symptoms and diseases are at the extremes of dimensions. Despite these new dimensional proposals, classifications and third-person approach have shown limitations. Their extraordinary evolution nevertheless underlines the contributions of developmental and psychodynamic frameworks. Developmental contributions have made it possible to evolve from disorders centered on a first-person perspective. Complementarily to the first-person/third-person perspectives, we advocate a second-person perspective, based on intersubjectivity. This perspective reverses the intuitive trend to focus our interventions on the most specific symptoms and syndromes, and advocates instead interventions on a "p" general factor that are both generalized and highly targeted. The implications are (1) to intervene as early as possible, (2) to base the definition of our therapeutic targets on an intersubjective perspective, (3) to identify and enhance children's and parents' strengths. These empirically informed directions are not in the current mainstream of psychopathology frameworks, and need to be developed., Competing Interests: The author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Ouss.)

Objective: Improving or maintaining the quality of life of the family of children treated with long term continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) is a major concern; but studies are scarce. The aim of the study was to evaluate the impact of long term CPAP or NIV in children on anxiety, depression, quality of sleep, and quality of life of their parents., Methods: Validated questionnaires evaluating anxiety and depression (hospital anxiety and depression scale), sleep quality (Pittsburgh sleep quality index), daytime sleepiness (Epworth sleepiness scale), and parents' quality of life (PedsQL family impact module) were completed by parents of children who were started on CPAP/NIV before (M0) and after 6-9 months (M6) of treatment., Results: The questionnaires of 36 parents (30 mothers, 6 fathers) of 31 children were analyzed. For the entire group, no significant change was observed in anxiety, depression, sleep quality, daytime sleepiness, and quality of life between M0 and M6. When analyzing questionnaire class changes between M0 and M6: anxiety was relieved in 23% of parents and worsened in 29%, depression was relieved in 14% and worsened in 20%, sleep quality improved in 43% and worsened in 27%, sleepiness improved in 26% and worsened in 17%, with no change in the other parents., Conclusion: Long term CPAP/NIV in children had no significant effect on parents' anxiety, depression, sleep quality, and quality of life., (© 2023 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.)

Objective: To study longitudinal changes in tuber and whole-brain perfusion in children with tuberous sclerosis complex (TSC) using arterial spin labeling (ASL) perfusion MRI and correlate them with pathological EEG slow wave activity and neurodevelopmental outcomes., Methods: Retrospective longitudinal cohort study of 13 children with TSC, 3 to 6 serial ASL-MRI scans between 2 months and 7 years of age (53 scans in total), and an EEG examination performed within 2 months of the last MRI. Tuber cerebral blood flow (CBF) values were calculated in tuber segmentation masks, and tuber:cortical CBF ratios were used to study tuber perfusion. Logistic regression analysis was performed to identify which initial tuber characteristics (CBF value, volume, location) in the first MRI predicted tubers subsequently associated with EEG slow waves. Whole-brain and lobar CBF values were extracted for all patient scans and age-matched controls. CBF ratios were compared in patients and controls to study longitudinal changes in whole-brain CBF., Results: Perfusion was reduced in tubers associated with EEG slow waves compared with other tubers. Low tuber CBF values around 6 months of age and large tuber volumes were predictive of tubers subsequently associated with EEG slow waves. Patients with severe developmental delay had more severe whole-brain hypoperfusion than those with no/mild delay, which became apparent after 2 years of age and were not associated with a higher tuber load., Conclusions: Dynamic changes in tuber and brain perfusion occur over time. Perfusion is significantly reduced in tubers associated with EEG slow waves. Whole-brain perfusion is significantly reduced in patients with severe delay., Key Points: • Tubers associated with EEG slow wave activity were significantly more hypoperfused than other tubers, especially after 1 year of age. • Larger and more hypoperfused tubers at 6 months of age were more likely to subsequently be associated with pathological EEG slow wave activity. • Patients with severe developmental delay had more extensive and severe global hypoperfusion than those without developmental delay., (© 2022. The Author(s), under exclusive licence to European Society of Radiology.)

Objectives: To assess practices of French psychiatrists regarding their management of children and adolescents with suicidal behaviors, focusing on the use of a separation protocol in which the youths are separated from their relatives., Methods: In 2017, we conducted an online cross-sectional survey of French psychiatrists caring for children and adolescents. Participants were asked to describe their practice of a separation protocol in children and adolescents admitted for suicidal behavior. Our main analysis followed a descriptive approach. We also explored whether participant characteristics were associated with the use of a separation protocol., Results: The response rate was 218/2403 (9,1%); 57.9 % of respondents worked in a University hospital, and 60% of respondents reported routinely hospitalizing children. A separation protocol was set up by 91.1% of survey participants (systematically 39.6%, on a case-by-case basis 51.5%). The mean age from which a separation protocol was indicated was above 11 years; 64% of participants reported a separation period of ≤ 48 h. The most common (87%) criterion cited for establishing a separation period was family relationship difficulties. The most common (80.9%) reason to justify the use of a separation protocol was to allow a better clinical assessment. Exploratory analyses did not identify any participant characteristics associated with the use of a separation protocol ( p > 0.2 for all)., Conclusion: The use of a separation protocol in children and adolescents admitted for suicidal behavior is a widespread practice in France, despite the deprivation of liberty it implies. This raises the question of the relevance and usefulness of such a practice., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Payet, Bonfils, Ouss, Fourcade, Touati-Pellegrin, Golse, Cohen and Woestelandt.)

Aim: The aim of the study was to assess the emotional and behavioural functioning of siblings of children treated with long term non-invasive ventilation (NIV)., Methods: Parents of children treated with NIV completed the Child Behaviour Checklist and a qualitative questionnaire for each sibling, aged 1.5-18 years old., Results: The parents of 49 ventilated children were questioned about 79 siblings. For the siblings aged 1.5-5, mean total T score was 57 ± 22 (range 28-92), and five siblings (31%) were in the clinical range. For the siblings aged 6-18, mean total T score was 49 ± 12 (range 26-71), and six siblings (10%) were in the clinical range. Siblings felt responsible for their affected sibling (31%) and involved with his/her illness (52%), with 31% being worried about him/her. A change in behaviour was observed in 19% of the siblings since the initiation of NIV; 26% were impacted by the use of the NIV device., Conclusions: The majority of siblings of children treated with NIV do not present significant emotional and behavioural problems. They feel deeply responsible for their affected sibling and involved in his/her illness and treatment, highlighting the importance to involve the siblings in the care of the affected child., (© 2021 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Purpose: To evaluate emotional distress and prenatal attachment throughout a subsequent pregnancy after Termination of Pregnancy (TOP) for fetal abnormality., Methods: Observational study, in a French Tertiary Maternity., Population: 25 women in a subsequent pregnancy after a medical termination of pregnancy for foetal abnormality, 18-year-old and older. Prenatal Interviews at 20 Gestationnal weeks (GW), 27 GW and 35 GW and Postnatal at 3 months and at each time self-administered questionnaires of anxiety, post-traumatic stress syndrome (PCLS) depressive symptoms (EPDS), prenatal attachment (PAI) and Perinatal Grief Scale (PGS)., Results: Pregnancy onset, i.e. before 20 GW, showed increased prevalence of anxiety (16/23, 66.7%), depression (7/23, 30.4%) and post-traumatic stress symptoms (4/16, 25%). Total score on PGS is higher in onset of pregnancy than in the third trimester (p = 0.005). Prenatal attachment was lower during early pregnancy (p = 0.003) and correlated inversely with grief intensity (p = 0.022). During late pregnancy, emotional symptoms decrease, and prenatal attachment stopped increase positively, specifically among women whose foetal abnormality in previous pregnancies were diagnosed late, at an average of 25 GW., Conclusion: This research shows the specific dynamics of pregnancies following TOP and highlights the necessity for early prenatal psychological support. One should also pay special attention to prenatal attachment during late pregnancy even after knowing that the fetus is healthy., Competing Interests: Declaration of Competing Interest Authors Declare no conflict of interest, (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

This retrospective observational study conducted in Necker Hospital for Sick Children, France (January 2018-June 2020) evaluated a potential temporal association between admissions for suicide behaviours in children and adolescents and the national COVID-19 lockdown (March-May 2020). During the study period, 234 patients were admitted for suicide behaviours (28% male; mean age 13.4 years). Using Poisson regression, we found a significant decrease in the incidence of admissions for suicide behaviour during the lockdown (adjusted incidence rate ratio: 0.46; 95% CI 0.24 to 0.86). This association might result from reduced help-seeking and decreased hospital admission rates during the lockdown, as well as cognitive and environmental factors. Further multicentre studies should be conducted to confirm these findings and investigate whether a compensatory rise in admissions for suicide behaviour occurred in the postlockdown period., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: The aim of this study was to investigate attachment behavior in a population of infants with infantile spasms (ISs) using the Strange Situation Procedure (SSP) and to explore factors associated with the infants' attachment behavior., Methods: The SSP was assessed in a population of 29 children with ISs during the second year of life. In mothers, we assessed anxiety, depression, maternal emotions, and perception of the temperament of the child, and sociodemographic characteristics. In children, we assessed epilepsy characteristics, response to antiepileptic drugs (AEDs) at the time of the SSP, and the child's outcome at 3 years of age, in terms of intellectual disability (ID), and autism spectrum disorder (ASD)., Results: Insecure attachment was higher than in the general population (68% versus 32%). It was associated with maternal anxiety, sadness, and maternal representation of the child at 12 months but with none of the child characteristics including ID, ASD, response to AEDs, or ISs etiology., Significance: Nonspecific dimensions were more important than disease characteristics for the infants' attachment behavior. In conclusion, we propose that interventions targeting mother-child interaction could prevent attachment insecurity and the developmental consequences of early epilepsy., Competing Interests: Declaration of competing interest None., (Copyright © 2020 Elsevier Inc. All rights reserved.)

The objective of the study was to examine the process of mother to infant trauma transmission among traumatized mothers in humanitarian contexts. We investigated the impact of mothers' post-traumatic stress disorder symptoms on the quality of the dyadic interaction by conducting a microanalysis of mother-infant interactions at specific moments when trauma was recalled, compared to more neutral moments. Twenty-four mother-infant dyadic interactions of traumatized mothers and children aged from 1.5 to 30 months Central Africa, Chad, and Cameroon were videotaped during three sequences: a neutral initial session (baseline) exploring mothers' representations of the infant and of their bonding; a second sequence, "the traumatic narration," in which mothers were asked to talk about the difficult events they had experienced; and a third sequence focusing on a neutral subject. Three minutes of each sequence were coded through a specific grid for microanalysis [based on the scales developed at Bobigny Faculty of Medicine and the work of (1)], according to different communication modalities (touch, visual, and vocal), for both the mother and the child. Impact of traumatic event (IES-R), the level of depression and anxiety (HAD) were investigated in order to have a holistic understanding of the trauma transmission mechanism. The data analysis highlighted significant differences in mothers, children and their interaction during the "traumatic narration": mothers touched and looked at the infant less, looked more absent and smiled less, and looked less at the interviewer; infants looked less at the interviewer, and sucked the breast more. The mother-child interaction "infant self-stimulation-mother looks absent" and "Infant sucks the breast-mother looks absent" occurred more often during the mothers' traumatic narrations. The "absence" of the mother during trauma recall seems to have repercussions on infants' behavior and interaction; infants show coping strategies that are discussed. We found no significant associations between interaction and infant gender and age, the severity of traumatic experience, mothers' depression and anxiety symptoms, and the country of residence. The results of the microanalysis of interaction can shed light on the fundamental role of intermodal exchanges between mother and infant in trauma transmission during mothers' trauma reactivation., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2020 Dozio, Feldman, Bizouerne, Drain, Laroche Joubert, Mansouri, Moro and Ouss.)

Aim: To examine how children with sleep-disordered breathing express their own sleep through drawing., Methods: Children hospitalised for a sleep study in a sleep laboratory of a tertiary hospital were asked to draw a human figure and themselves while asleep. Characteristics of the two drawings were analysed and compared along with a descriptive analysis of some drawings., Results: Children with sleep-disordered breathing and an associated disorder, n = 34, age 5-11 years, participated in the study. The size of the human figure, the colours used, the orientation of the sheet, the type of drawing strokes and the objective quality of the drawing were comparable between the two drawings. On the sleep drawing, 71% of the children drew a bed, 15% drew themselves asleep, 19% represented snoring and 12% night elements. Sixty-two per cent of the children preferred the human drawing to the sleep drawing. A descriptive analysis of 12 drawings showed the influence of the associated disorder on the two drawings., Conclusion: This study showed how the associated disease of children with sleep-disordered breathing infiltrated their imaginary life. The sleep drawing gave useful information about representation, fears and wishes in relation to the associated disease and the child's sleeping., (© 2020 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Automated behavior analysis are promising tools to overcome current assessment limitations in psychiatry. At 9 months of age, we recorded 32 infants with West syndrome (WS) and 19 typically developing (TD) controls during a standardized mother-infant interaction. We computed infant hand movements (HM), speech turn taking of both partners (vocalization, pause, silences, overlap) and motherese. Then, we assessed whether multimodal social signals and interactional synchrony at 9 months could predict outcomes (autism spectrum disorder (ASD) and intellectual disability (ID)) of infants with WS at 4 years. At follow-up, 10 infants developed ASD/ID (WS+). The best machine learning reached 76.47% accuracy classifying WS vs. TD and 81.25% accuracy classifying WS+ vs. WS-. The 10 best features to distinguish WS+ and WS- included a combination of infant vocalizations and HM features combined with synchrony vocalization features. These data indicate that behavioral and interaction imaging was able to predict ASD/ID in high-risk children with WS.

Background: Trimethylaminuria (TMAU) is a metabolic disorder characterized by the excessive excretion of the malodorous compound trimethylamine (TMA). The diagnosis of TMAU is challenging because this disorder is situated at the boundary between biochemistry and psychiatry. Here, we used nuclear magnetic resonance spectroscopy to assess TMAU in 13 patients. We also sequenced the FMO3 gene in 11 of these patients. Treatment with vitamin B2 was prescribed., Results: Two patients (aged 3 and 9 years at the initial consultation) had a particularly unpleasant body odor, as assessed by their parents and the attending physicians. The presence of high urine TMA levels confirmed the presence of a metabolic disorder. The two (unrelated) children carried compound heterozygous variants in the FMO3 gene. In both cases, vitamin B2 administration decreased TMA excretion and reduced body odor. The 11 adults complained of an unpleasant body odor, but the physicians did not confirm this. In all adult patients, the urine TMA level was within the normal range reported for control (non-affected) subjects, although two of the patients displayed an abnormally high proportion of oxidized TMA. Seven of the 9 tested adult patients had a hypomorphic variant of the FMO3 gene; the variant was found in the homozygous state, in the heterozygous state or combined with another hypomorphic variant. All 11 adults presented a particular psychological or psychiatric phenotype, with a subjective perception of unpleasant odor., Conclusions: The results present the clinical and biochemical data of patients complaining of unpleasant body odor. Contrary to adult patients, the two children exhibited all criteria of recessively inherited trimethylaminuria, suspected by parents in infancy. B2 vitamin treatment dramatically improved the unpleasant body odor and the ratio of TMA/Cr vs TMAO/Cr in the urine in the children. Other patients presented a particular psychological or psychiatric phenotype.

Objective: We aimed to assess a cohort of young patients with Dravet syndrome (DS) for intellectual disability (ID) and autism spectrum disorder (ASD) using standardized tools and parental questionnaires to delineate their specific profiles., Methods: We included 35 patients with DS aged 24 months to 7 years, excluding patients with a developmental age (DA) <18 months (n = 5). We performed specific tests adapted for ID (Psychoeducational Profile, Third Edition [PEP-3]), in addition to the Child Development Inventory (CDI) and Vineland Adaptive Behavior Scales, Second Edition (VABS-II) questionnaires. We used 2 standardized tools for ASD: the Autism Diagnostic Observation Schedule, Second Edition (ADOS-2) and the Autism Diagnostic Interview-Revised (ADI-R). We compared the with parental questionnaires and the VABS-II, and with ASD characteristics., Results: PEP-3 subscales showed pathologic development in all but one patient (97%): ID in 23 of 30 (77%), and borderline cognitive functioning in 6 of 30 (22%). Eleven patients (39%) had ASD and 2 (7%) had a Social Communication Disorder (SCD) diagnosis. We found no difference between PEP-3 and CDI categorization except for fine motor skills. We found significant negative correlations between ADOS-2 and PEP-3 for the majority of scores. For patients aged older than 50 months, 2 groups emerged (ASD/no ASD) with significant difference in DA. The logistic regression for ASD diagnosis explained by VABS-II showed a significant effect for Socialization, Motor Skills, and Adaptive Behavior., Significance: We found a high prevalence of ID in patients with DS. ID is characterized by expressive and comprehensive communication deficits in addition to visuospatial difficulties. ASD showed a specific profile with a relative preservation of social skills, emphasizing a possible underdiagnosis. Parental questionnaires can provide a good assessment of cognitive profile and might allow the difficulty of addressing cognitive scales in DS to be overcome. The profile of ID and ASD should help to establish early adapted rehabilitation programs and emphasizes the global need for care beyond seizures in DS and other developmental epileptic encephalopathies., Competing Interests: None of the authors has any conflict of interest to disclose. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.

Characteristic preterm EEG patterns of "Delta-brushes" (DBs) have been reported in the temporal cortex following auditory stimuli, but their spatio-temporal dynamics remains elusive. Using 32-electrode EEG recordings and co-registration of electrodes' position to 3D-MRI of age-matched neonates, we explored the cortical auditory-evoked responses (AERs) after 'click' stimuli in 30 healthy neonates aged 30-38 post-menstrual weeks (PMW). (1) We visually identified auditory-evoked DBs within AERs in all the babies between 30 and 33 PMW and a decreasing response rate afterwards. (2) The AERs showed an increase in EEG power from delta to gamma frequency bands over the middle and posterior temporal regions with higher values in quiet sleep and on the right. (3) Time-frequency and averaging analyses showed that the delta component of DBs, which negatively peaked around 550 and 750 ms over the middle and posterior temporal regions, respectively, was superimposed with fast (alpha-gamma) oscillations and corresponded to the late part of the cortical auditory-evoked potential (CAEP), a feature missed when using classical CAEP processing. As evoked DBs rate and AERs delta to alpha frequency power decreased until full term, auditory-evoked DBs are thus associated with the prenatal development of auditory processing and may suggest an early emerging hemispheric specialization.

Aim: We evaluated the impact that having a child with sleep-disordered breathing had on their parents, including their own sleep quality., Methods: Questionnaires were completed by 96 parents of 86 children referred for a sleep study or control of continuous positive airway pressure (CPAP) or noninvasive ventilation (NIV) at the sleep laboratory of the Necker Hospital, Paris, France, between October 2015 and January 2016. The questionnaires evaluated anxiety and depression, family functioning, the parents' quality of life, daytime sleepiness and sleep quality., Results: The children had a mean age of seven ±five years and most of the responses (79%) came from their mothers. These showed that 26% of parents showed moderate-to-severe anxiety, 8% moderate-to-severe depression, 6% complex family cohesion, 59% moderate-to-severe daytime sleepiness and 54% poor sleep quality. Anxiety was higher in mothers than in fathers (p < 0.001). The questionnaire scores did not differ according to the child's age, the results of the sleep studies or the CPAP or NIV treatment. The symptoms seem to be more commonly related to the child's underlying disease than their sleep-disordered breathing., Conclusion: The parents of children referred to a sleep laboratory reported frequent anxiety, daytime sleepiness and poor sleep quality., (©2018 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Propionic acidemia is the result of a deficiency in propionyl-CoA carboxylase activity. Chronic neurologic and cognitive complications frequently occur, but the psychiatric evolution of the disorder is not well documented. We conducted a pedopsychiatric evaluation of 19 children, adolescents and young adults, aged between 2 and 25 years, using ADI-R, CARS-T, as well as ADOS when autism spectrum disorder was suspected. Previous psychometric examinations were also taken into consideration. Thirteen patients had an IQ < 80. Two patients presented with autism and two additional patients with other autism spectrum disorders. Five patients did not fulfill diagnostic criteria for autism spectrum disorder but showed difficulties indicative of a broader autism phenotype (BAP). Four other patients had severe anxiety manifestations related to their disease. Two patients presented with acute psychotic episodes. The number of decompensations in the first 3 years of life was lower in patients with autism spectrum disorder or related symptoms. These patients were also older when they were assessed (median age of 15 years old versus 11 years old). There was no significant correlation between 3-hydroxypropionate levels during the first 6 years of life and autism spectrum disorder diagnosis. In conclusion, autism spectrum disorder is frequent in patients with propionic acidemia. These patients should undergo in-depth psychiatric evaluation and be screened for autism spectrum disorder. Further studies are needed to understand the underlying mechanisms.

Highlights The kinematics of hand movements (spatial use, curvature, acceleration, and velocity) of infants with their mothers in an interactive setting are significantly associated with age in cohorts of typical and at-risk infantsdiffer significantly at 5-6 months of age, depending on the context: relating either with an object or a person.Environmental and developmental factors shape the developmental trajectories of hand movements in different cohorts: environment for infants with VIMs; stage of development for premature infants and those with West syndrome; and both factors for infants with orality disorders.The curvature of hand movements specifically reflects atypical development in infants with West syndrome when developmental age is considered. We aimed to discriminate between typical and atypical developmental trajectory patterns of at-risk infants in an interactive setting in this observational and longitudinal study, with the assumption that hand movements (HM) reflect preverbal communication and its disorders. We examined the developmental trajectories of HM in five cohorts of at-risk infants and one control cohort, followed from ages 2 to 10 months: 25 West syndrome (WS), 13 preterm birth (PB), 16 orality disorder (OD), 14 with visually impaired mothers (VIM), 7 early hospitalization (EH), and 19 typically developing infants (TD). Video-recorded data were collected in three different structured interactive contexts. Descriptors of the hand motion were used to examine the extent to which HM were associated with age and cohort. We obtained four principal results: (i) the kinematics of HM (spatial use, curvature, acceleration, and velocity) were significantly associated with age in all cohorts; (ii) HM significantly differed at 5-6 months of age in TD infants, depending on the context; (iii) environmental and developmental factors shaped the developmental trajectories of HM in different cohorts: environment for VIM, development for PB and WS, and both factors for OD and; (iv) the curvatures of HM showed atypical development in WS infants when developmental age was considered. These findings support the importance of using kinematics of HM to identify very early developmental disorders in an interactive context and would allow early prevention and intervention for at-risk infants.

This is the second of three papers that summarize the second symposium on Transition in Epilepsies held in Paris in June 2016. This paper addresses the outcome for some particularly challenging childhood-onset epileptic disorders with the goal of recommending the best approach to transition. We have grouped these disorders in five categories with a few examples for each. The first group includes disorders presenting in childhood that may have late- or adult-onset epilepsy (metabolic and mitochondrial disorders). The second group includes disorders with changing problems in adulthood (tuberous sclerosis complex, Rett syndrome, Dravet syndrome, and autism). A third group includes epilepsies that change with age (Childhood Absence Epilepsy, Juvenile Myoclonic Epilepsy, West Syndrome, and Lennox-Gastaut syndrome). A fourth group consists of epilepsies that vary in symptoms and severity depending on the age of onset (autoimmune encephalitis, Rasmussen's syndrome). A fifth group has epilepsy from structural causes that are less likely to evolve in adulthood. Finally we have included a discussion about the risk of later adulthood cerebrovascular disease and dementia following childhood-onset epilepsy. A detailed knowledge of each of these disorders should assist the process of transition to be certain that attention is paid to the most important age-related symptoms and concerns., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Conversion disorder in childhood. Conversion disorder in childhood exists and remains a complex entity whose clinics and psychopathology vary according to the chosen definition or to the theoretical framework. At present, this pathology remains the source of numerous diagnostic questions and nosographic confusions in current pediatric practice. This article tries to define and describe in a synthetic way the specificities of child conversion disorder, and the processes underlying the symptomatic outbreak. Today, there is no consensus on the management of these young patients, but we will try to identify the main features for a comprehensive therapeutic approach., Competing Interests: Les auteurs déclarent n’avoir aucun lien d’intérêts.

Suicide remains one of the leading causes of death among young people, and suicidal ideation and behavior are relatively common in healthy and clinical populations. Suicide risk in childhood and adolescence is often approached from the perspective of nosographic categories to which predictive variables for suicidal acts are often linked. The cascading effects resulting from altered clock genes in a pediatric population could participate in biological rhythm abnormalities and the emergence of suicide attempts through impaired regulation of circadian rhythms and emotional states with neurodevelopmental effects. Also, early trauma and stressful life events can alter the expression of clock genes and contribute to the emergence of suicide attempts. Alteration of clock genes might lead to desynchronized and abnormal circadian rhythms impairing in turn the synchronization between external and internal rhythms and therefore the adaptation of the individual to his/her internal and external environment with the development of psychiatric disorders associated with increased risk for suicide attempts., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Introduction: Autism features and various degrees of cognitive deficit are reported in patients with PCDH-19 mutations and epilepsy. Autism spectrum disorder (ASD) and, often, cognitive profile are usually assessed clinically. We studied autism phenotype and cognitive outcome in a series of patients using standardized tools for development and ASD. We aimed to describe the phenotype of ASD in this series and to understand whether ASD is strictly linked to intellectual disability (ID) or is present as a comorbidity., Methods: Eight females aged 5 to 17years old with PCDH-19 mutations and epilepsy were recruited. For ASD diagnosis, the Autism Diagnostic Interview - Revised (ADI-R) and the Autism Diagnosis Observation Schedule (ADOS) were administered. Patients underwent a neuropsychological examination with tests measuring global intellectual efficiency (WPPSI-III and WISC-IV), language, and executive and social cognition abilities. Parental adaptive behavioral questionnaires were also obtained (VABS, CBCL, and BRIEF)., Results: Six out of eight patients presented with ASD and ID. Two patients had neither ASD nor ID, and both had the latest age of onset for their epilepsy. All cognitive functions were deficient, but theory-of-mind abilities compared to other cognitive features were even impaired. Features of ASD lacked major repetitive and stereotyped behaviors and show some differences with the classical ASD features related to ID., Conclusion: Our results show a large spectrum of ID and a very high rate of ASD in patients with epilepsy and PCDH-19 mutations. Autism spectrum disorder seems to be a genuine comorbidity, more than a consequence of ID. It highlights the importance of standardized psychiatric and cognitive evaluation in order to establish a tailored rehabilitation program., (Copyright © 2016 Elsevier Inc. All rights reserved.)

This paper aims to describe current questions concerning conversive disorders among children and adolescents. We first describe prevalence and clinical characteristics of these. Many unresolved questions remain. Why do patients show excess, or loss of function? Attachment theory offers a relevant framework to answer this question. Does neurobiology of conversion disorders shed light on conversive processes? Current neurobiological research paradigms focus on the symptom, trying to infer processes, instead of proposing paradigms that test theoretical hypotheses. The most convincing theoretical framework that has already proposed a coherent theory of conversion is a psychodynamic one, which has not yet been tested with neurobiological paradigms. The interest of studying child and adolescent conversive disorders is to provide a means to more deeply investigate the two challenges we face: theoretical, and clinical ones. It provides the opportunity to access a pathopsychological process at its roots, not yet hidden by many defensive, rationalizing attitudes, and to better explore environmental features. We propose a "complementarist" model, which allows the combination of different approaches (neural, cognitive, environmental, attachment, intra-psychic) and permits proposal of different levels of therapeutic targets and means., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

West syndrome (WS) is a rare epileptic encephalopathy with early onset and a high risk of autistic outcome. The PréAut grid assesses this risk following WS onset by taking into account synchrony and emotion in interactions and by evaluating the baby's active desire to engage in pleasant interactions (especially the infant's early active behaviors that encourage being gazed at or kissed by the mother or to share joy with her). We followed a sample of 25 WS patients prospectively from disease onset and assessed whether the PréAut grid before 9 months, and the checklist for autism in toddlers (CHAT) at 18 and 24 months predicted autism or intellectual disability (ID) outcomes at 4 years. We found that the PréAut grid at 9 months (sensitivity = 0.83; specificity = 1) had similar prediction parameters as the CHAT at 18 months (sensitivity = 0.90; specificity = 0.83) and 24 months (sensitivity = 0.92; specificity = 1). WS patients with a positive PréAut screening at 9 months had a risk of having autism or ID at 4 years, which is 38 times that of children with a negative PréAut grid [OR = 38.6 (95 % CI 2.2-2961); p = 0.006]. We conclude that the PréAut grid could be a useful tool for the early detection of autism or ID risk in the context of WS. Further research is needed to assess the PréAut grid in other contexts (e.g. infants at high-risk for non-syndromic autism).

This study focused on parents' satisfaction with the special education and care services proposed to their child with autism spectrum disorders (ASD). Data were collected in three regions of France, using a questionnaire designed for the purpose of this study. Among the 530 families contacted, 212 filled in the questionnaire (response rate = 40.8%). Results showed that parents were globally satisfied with providers' involvement and motivation, but they felt they were not involved enough in their child's individualized program, that communication with providers was insufficient and that the services lacked ASD's specific tools and interventions. Among all families interviewed, parents of adolescents were the most unsatisfied and we hypothesized that this could be due to the specific issues regarding developmental changes and concern about the future at this period of life. Congruently with the literature, variables related to parental overall satisfaction were a regular communication with professionals, a specific, regularly updated individual program in which parents are associated, and specialized tools and interventions. The implications of these findings are discussed as well as future directions for clinicians to improve service delivery and allow the persons with ASD and their families to be more involved in the services.

Research has shown a high rate of autism spectrum disorders among very low birth weight children over the past decade. This paper proposes a literature review on this topic. Two generations of research have followed one another. The first retrospective studies found a high rate of ASD among premature babies. The second generation of prospective studies underlined and relativized this risk. Prospective research using screening tools (M-CHAT) have found around 20 % ASD, whereas 2 studies assessing the actual diagnosis found 5 % and 8 % ASD, 10 to 12 times more than in the general population. A number of hypotheses have been put forward to explain these high rates of ASD: sensory impairment associated with prematurity, white matter abnormalities, and cerebellar impairment. The authors propose complex models that take into account neurological deficits and the effects of perinatal events on interactive dynamics between infants and their caregivers. These models aim to allow suitable prevention and care for premature children with autism, a heavy additional handicap., (Copyright © 2012. Published by Elsevier SAS.)

Providing abandoned children the necessary medical and psychological care as possible after their institutionalization may minimize developmental delays. We describe psychomotor development in infants admitted to an orphanage in Khartoum, Sudan, assessed at admission and over an 18-month follow-up. Psychological state and psychomotor quotients were determined using a simplified Neonatal Behavior Assessment Scale (NBAS), the Brunet-Lezine and Alarm distress baby (ADBB) scale. From May-September 2005, 151 children were evaluated 2, 4, 9, 12 and 18 months after inclusion. At admission, ~15% of children ≤1 month had a regulation impairment according to the NBAS, and 33.8% presented a distress state (ADBB score >5). More than 85% (129/151) recovered normal psychomotor development. The results of the program reinforce the importance of early detection of psychological disorders followed by rapid implementation of psychological case management to improve the development of young children in similar institutions and circumstances.

Our model of psychotherapy for patients with brain lesions is based on an integrative approach of psychobehavioral symptoms, especially from the neuropsychological and psychodynamic perspectives. Adjustment of technical modalities and aims of psychoanalytical therapy is required for these patients. The analysis of the influence of cognitive disorders on transference and contre-transference plays a major role, including the role of procedural processes in changes in the intersubjective relationship between the patient and the therapist. Two vignettes are presented to illustrate our model, which respects the integrity of the cognitive and psychodynamic approaches and can be implemented by only one therapist, using alternatively each lecture, or by a working team bringing to light the different aspects of the same symptom.

This paper aims to develop the rational to support why and how we should link neuroscience and psychoanalysis. Many of these points are derived from child development and child psychiatry. Neuroscience investigates developmental questions in a different way than psychoanalysis, while psychoanalysis itself has shifted towards new developmental paradigms. The rapprochement between neuroscience and psychoanalysis allows a new understanding of some concepts, including embodiment of mind, consciousness and attachment. The "double reading" paradigm allows a better understanding of symptomatic configurations. Linking neuroscience and psychoanalysis may improve treatments and result in new experimental neuroscientific paradigms involving changing the research object, changing the state of the research object, and investigating the structural changes in the brain following psychotherapy. The last aim is to create an epistemology of the articulation between the theoretical frameworks through phenomenology, "complementarism" and neuropsychoanalysis. We argue that it is necessary for clinicians to be aware of the advancements in each field. This is not only an epistemological question; we assume that new findings in neuroscience will change the way psychoanalysts think and approach treatment of their patients. We hope the present research will contribute to change the way that neuroscientists think and will provide new options to their set of experimental paradigms., (Copyright © 2010 Elsevier Ltd. All rights reserved.)

This work presents the validation of a French version of the emotional self rating scale by Izard (Differential Emotion Scale). This scale is based on the theory of the Discrete Emotions which proposes the existence of ten fundamental emotions. This self-rating scale consists of thirty emotional adjectives (three for each emotion). The validation was done by using a non inductive method (by asking the subject to describe his present emotional state) with 84 control subjects. It shows that this version is valid. Main results are first the binarisation of the emotions (positive and negative), then the existence of complex factors with psychopathological meaning after factorial analysis. These results are in agreement with a theory of expressive emotional levels more than with the theory of discrete emotions in psychopathology.