Understanding neurodevelopmental trajectories and behavioral profiles in SCN1A-related epilepsy syndromes.

BACKGROUND: A pathogenic variant in SCN1A can result in a spectrum of phenotypes, including Dravet syndrome (DS) and genetic epilepsy with febrile seizures plus (GEFS + ) syndrome. Dravet syndrome (DS) is associated with refractory seizures, developmental delay, intellectual disability (ID), motor impairment, and challenging behavior(1,2). GEFS + is a less severe phenotype in which cognition is often normal and seizures are less severe. Challenging behavior largely affects quality of life of patients and their families. This study describes the profile and course of the behavioral phenotype in patients with SCN1A-related epilepsy syndromes, explores correlations between behavioral difficulties and potential risk factors. METHODS: Data were collected from questionnaires, medical records, and semi-structured interviews. Behavior difficulties were measured using the Adult/Child Behavior Checklist (C/ABCL) and Adult self-report (ASR). Other questionnaires included the Pediatric Quality of Life Inventory (PedsQL), the Functional Mobility Scale (FMS) and the Sleep Behavior Questionnaire by Simonds & Parraga (SQ-SP). To determine differences in behavioral difficulties longitudinally, paired T-tests were used. Pearson correlation and Spearman rank test were used in correlation analyses and multivariable regression analyses were employed to identify potential risk factors. RESULTS: A cohort of 147 participants, including 107 participants with DS and 40 with genetic epilepsy with febrile seizures plus (GEFS + ), was evaluated. Forty-six DS participants (43.0 %) and three GEFS + participants (7.5 %) showed behavioral problems in the clinical range on the A/CBCL total problems scale. The behavioral profile in DS exists out of withdrawn behavior, aggressive behavior, and attention problems. In DS patients, sleep disturbances (ß = 1.15, p < 0.001) and a lower age (ß = -0.21, p = 0.001) were significantly associated with behavioral difficulties. Between 2015 and 2022, behavioral difficulties significantly decreased with age (t = -2.24, CI = -6.10 - -0.15, p = 0.04) in DS participants aging from adolescence into adulthood. A decrease in intellectual functioning (ß = 3.37, p = 0.02) and using less antiseizure medications in 2022 than in 2015, (ß = -1.96, p = 0.04), were identified as possible risk factors for developing (more) behavioral difficulties. CONCLUSIONS: These findings suggest that, in addition to epilepsy, behavioral difficulties are a core feature of the DS phenotype. Behavioral problems require personalized management and treatment strategies. Further research is needed to identify effective interventions.

Exploring individual parent-to-parent support interventions for parents caring for children with brain-based developmental disabilities: A scoping review.

BACKGROUND: Brain-based developmental disabilities (BBDDs) comprise a large and heterogeneous group of disorders including autism, intellectual disability, cerebral palsy or genetic and neurodevelopmental disorders. Parents caring for a child with BBDD face multiple challenges that cause increased stress and high risk of mental health problems. Peer-based support by fellow parents for a various range of patient groups has shown potential to provide emotional, psychological and practical support. Here, we aim to explore existing literature on individual peer-to-peer support (iP2PS) interventions for parents caring for children with BBDD with a view to (1) explore the impact of iP2PS interventions on parents and (2) identify challenges and facilitators of iP2PS. METHOD: An extensive literature search (January 2023) was performed, and a thematic analysis was conducted to synthesize findings. RESULTS: Fourteen relevant articles revealed three major themes regarding the impact of iP2PS on parents: (1) emotional and psychological well-being, (2) quality of life and (3) practical issues. Four themes were identified describing challenges and facilitators of iP2PS: (1) benefits and burden of giving support, (2) matching parent-pairs, (3) logistic challenges and solutions and (4) training and supervision of parents providing peer support. CONCLUSIONS: This review revealed that iP2PS has a positive impact on the emotional and psychological well-being of parents, as well as the overall quality of life for families caring for a child with a BBDD. Individual P2PS offers peer-parents an opportunity to support others who are facing challenges similar to those they have experienced themselves. However, many questions still need to be addressed regarding benefits of different iP2PS styles, methods of tailoring support to individual needs and necessity of training and supervision for peer support providers. Future research should focus on defining these components and evaluating benefits to establish effective iP2PS that can be provided as standard care practice for parents.

Parental Beliefs About the Motor Development of Dutch Infants Born Very Preterm: A Cohort Study.

PURPOSE: To explore the influence of preterm birth on parental beliefs about gross motor development and parents' supportive role in infants' motor development. METHODS: Prospective cohort study: Parents of infants born very preterm (VPT) (gestation ≤32 weeks, birth weight <1500 g, without perinatal complications) and parents of healthy infants born full-term (FT) completed the Parental Beliefs on Motor Development questionnaire. RESULTS: Questionnaires from 37 parents of infants born VPT, aged 3.5 to 7.5 months (corrected), and 110 parents of infants born FT, aged 3.5 months, were analyzed. Parents of infants born VPT believed stimulating motor development to be more important than parents of infants born FT (F = 5.22; P = .024; ηp2 = 0.035). Most parents of infants born VPT (82.4%) and FT (85.2%) acknowledged their role in supporting motor development. More parents of infants born VPT (41.2% vs 12.0%) believed they should follow their infant's natural developmental pace. CONCLUSION: Knowledge of parental beliefs and parents' supporting role in motor development is relevant for tailoring pediatric physiotherapists' interventions with families.

Challenging behavior in children and adolescents with Dravet syndrome: Exploring the lived experiences of parents.

BACKGROUND: Dravet syndrome (DS) is a monogenic syndrome associated with SCN1A mutations in the majority of patients and characterized by devastating epilepsy, that may be life-threatening. Aside from refractory seizures, core symptoms of DS include behavioral difficulties, developmental delay, cognitive impairment, and motor dysfunction. Previous DS research has mainly focused on epileptic seizures and pharmacological management and less on behavioral difficulties. This study aims to explore the lived experience of parents supporting a child with DS, with a focus on behavioral aspects. METHODS: We performed a qualitative study using focus groups and following the consolidated criteria for reporting qualitative research (COREQ) guidelines. We organized three focus groups with parents of children and adults with DS and used a pre-defined topic list of open questions, similar for each focus group to ensure comparability. The focus groups were video recorded, transcribed, and anonymized. Data were analyzed using an iterative coding process where codes were sorted into themes. Differences in coding among the researchers were discussed until a consensus was reached. RESULTS: In total, twenty parents (mothers only) participated in the study. The age of children with DS ranged between 3 to 22 years with a mean age of 11.8 years. A range of behavioral difficulties emerged from the thematic analysis. Overall, the most commonly mentioned behavioral difficulties were aggression, dangerous behavior, impulsivity, hyperactivity, routinized and compulsive habits. Our results showed different behavior per age group, with more externalizing behaviors such as aggression and impulsivity in children aged 3-13 years; and more internalizing behavior such as routinized and compulsive habits in adolescents and young adults (14-22 years). This results in a different kind of support these families need and should be acknowledged when in consult with a healthcare professional. Parents reported that challenging behavior was a source of stress and impacted negatively on their family's quality of life. Parents reported feeling alone in their search for solutions, and many explored options outside the traditional medical context. CONCLUSION: Our results suggest that the challenging behavior associated with DS leads to a huge burden of care. Healthcare professionals working with DS patients may need to develop shared decision-making strategies that take into account challenging behavior.

Factors associated with gross motor development from birth to independent walking: A systematic review of longitudinal research.

AIM: The aim of this study is to gain more insight into child and environmental factors that influence gross motor development (GMD) of healthy infants from birth until reaching the milestone of independent walking, based on longitudinal research. BACKGROUND: A systematic search was conducted using Scopus, PsycINFO, MEDLINE and CINAHL to identify studies from inception to February 2020. Studies that investigated the association between child or environmental factors and infant GMD using longitudinal measurements of infant GMD were eligible. Two independent reviewers extracted key information and assessed risk of bias of the selected studies, using the Quality in Prognostic Studies tool (QUIPS). Strength of evidence (strong, moderate, limited, conflicting and no evidence) for the factors identified was described according to a previously established classification. RESULTS: In 36 studies, six children and 11 environmental factors were identified. Five studies were categorized as having low risk of bias. Strong evidence was found for the association between birthweight and GMD in healthy full-term and preterm infants. Moderate evidence was found for associations between gestational age and GMD, and sleeping position and GMD. There was conflicting evidence for associations between twinning and GMD, and breastfeeding and GMD. No evidence was found for an association between maternal postpartum depression and GMD. Evidence for the association of other factors with GMD was classified as 'limited' because each of these factors was examined in only one longitudinal study. CONCLUSION: Infant GMD appears associated with two child factors (birthweight and gestational age) and one environmental factor (sleeping position). For the other factors identified in this review, insufficient evidence for an association with GMD was found. For those factors that were examined in only one longitudinal study, and are therefore classified as having limited evidence, more research would be needed to reach a conclusion.

Parents' experiences of VOICE: A novel support programme in the NICU.

BACKGROUND: Admission of an infant to a neonatal intensive care unit (NICU) is often a stressful experience for parents and can be associated with feelings of inadequacy to fulfil the desirable parental role. The values, opportunities, integration, control, and evaluation (VOICE) programme was developed to engage parents in care, to decrease stress, and to increase empowerment. AIM: To explore the experiences of parents regarding involvement in the VOICE programme during admission of their infant to the NICU. DESIGN: The VOICE programme includes at least five personal structured meetings between parents, nurses, and other health care professionals throughout the pathway from birth, NICU, and follow up. A qualitative design was adopted using semi-structured interviews. Interviews with 13 parents of 11 infants born at <27 weeks' gestational age were conducted: nine mothers and two couples of father and mother. Thematic analysis was deployed. RESULTS: The findings have been described in one overarching theme: "parental empowerment." Parents felt strengthened and were empowered in the development of their role as primary caretaker by the VOICE programme. The parental empowerment theme emerged from four related interpretive themes that were derived: (a) involvement in care, (b) personalized information and communication, (c) transition to a parental role, and (d) emotional support. CONCLUSION: The VOICE programme can be a structured approach used to implement family support in a NICU to empower parents to become a partner in the care of their infant and feel confident. RELEVANCE TO CLINICAL PRACTICE: This study encourages health care professionals to provide parental support through a structured intervention programme, which contributes to the empowerment of parents in the NICU and encouraged them to participate in care and decision-making.

The long-term effect of perinatal asphyxia on hippocampal volumes.

BACKGROUND: Hypoxic-ischemic encephalopathy (HIE) in term-born infants can lead to memory problems. The hippocampus is important for long-term episodic memory. The primary aim was to investigate the effect of HIE on hippocampal volumes in 9- to 10-year-old children. The secondary aim was to investigate the association between hippocampal volumes and previously found impaired memory and cognitive functions in the current cohort. METHODS: In total 26 children with mild HIE, 26 with moderate HIE, and 37 controls were included. The intelligence quotient (IQ) and memory were tested. A 3D-volumetric MRI was obtained. Brain segmentation was performed for hippocampal volumes and intracranial volume. The differences in hippocampal volumes, memory, and IQ between the groups were determined. Multivariable linear regression analyses were performed, including hippocampal volume as a percentage of intracranial volume as a dependent variable. RESULTS: Smaller hippocampal volumes were found in moderate HIE (p < 0.001), with a trend toward smaller volumes in mild HIE, compared to controls. In multivariable linear regression analysis, hippocampal volume as a percentage of intracranial volume was significantly associated with long-term visuospatial memory. CONCLUSION: Children with moderate HIE had smaller hippocampal volumes than controls, with a trend toward smaller volumes following mild HIE. Reduced hippocampal volumes were associated with poorer long-term visuospatial memory.

Behavior problems and health-related quality of life in Dravet syndrome.

OBJECTIVE: Behavior problems in Dravet syndrome (DS) are common and can impact the lives of patients tremendously. The current study aimed to give more insight into (1) the prevalence of a wide range of specific behavior difficulties and aspects of health-related quality of life (HRQoL) in patients with DS compared with the general population (gp) and patients with epilepsy without DS, (2) the relations between these behavior problems and different aspects of HRQoL, and (3) the associations between seizure frequency, cognitive impairment (CI), behavior problems, and HRQoL, based on a conceptual model. METHODS: One hundred and sixteen patients (aged between 2 and 67 years), affected by SCN1A-related seizures, were included in the study. Eighty-five were patients with DS, 31 were patients with epilepsy without DS. Behavior problems were measured using the Child/Adult Behavior Checklist (C/ABCL), HRQoL was measured using the Pediatric Quality of Life Inventory (PedsQL) Measurement Model. Other characteristics were obtained by clinical assessments, medical records, and semi-structured telephone interviews with parents. Comparisons between patients with DS, patients without DS, and the gp were calculated by the exact goodness of fit χ2 analyses, relations between subscales were analyzed using Pearson's correlations, and the conceptual model was tested in a path analysis. RESULTS: (1) Patients with DS show significantly more behavior problems compared with the gp and patients with epilepsy without DS. A total of 56.5% of patients with DS scored in the borderline and clinical ranges for total behavior problems. Problems with attention were most prevalent; 62.3% of patients with DS scored in the borderline and clinical ranges. Health-related quality of life was significantly lower for patients with DS compared with the gp and patients without DS. Physical and social functioning scores were especially low and decreased even more in the older age categories. (2) Problems with attention, aggression, and withdrawn behavior were most related to social functioning. Somatic problems and anxiety/depression were most related to emotional functioning. (3) Cognitive impairment and behavior problems were both independent predictors of poorer HRQoL in patients with DS, with behavior problems being the strongest predictor. Seizure frequency was only indirectly related to HRQoL, mediated by cognitive impairment. IMPLICATIONS: The high prevalence of behavior problems in DS and the significant impact on quality of life (QoL), independent of epilepsy-related factors, emphasize the need for active management and treatment of these problems and should be considered as part of the management plan.

Parental empowerment in paediatric rehabilitation: Exploring the role of a digital tool to help parents prepare for consultation with a physician.

BACKGROUND: To explore experiences of parents of children with disabilities using the WWW, roadmap, a tool to support them in exploring needs, finding information, and asking questions of professionals and to explore differences between parents who had used the WWW-roadmap to prepare for consultation with their rehabilitation physician and parents who had not. METHODS: In a sequential cohort study, we included 128 parents; 54 used the WWW-roadmap prior to consultation and 74 received care-as-usual. Both groups completed questionnaires after consultation, assessing empowerment, self-efficacy, parent and physician satisfaction, family centredness of care, and experiences using the tool. Additionally, 13 parents were interviewed. RESULTS: Parents who used the WWW-roadmap looked up more information on the Internet. No other differences between parents and physicians were found. In the interviews, parents said that the WWW-roadmap was a useful tool for looking up information, exploring and asking questions, and maintaining a comprehensive picture. CONCLUSION: Using the WWW-roadmap prior to consultation did not improve self-efficacy, satisfaction, or family centredness of care. Findings suggest positive experiences regarding factors determining empowerment, creating conditions for a more equal parent-physician relationship. The WWW-roadmap is useful for parents to explore their needs and find information, but more is needed to support empowerment in consultations.

Daily actions, challenges, and needs among Dutch parents while supporting the participation of their child with a physical disability at home, at school, and in the community: a qualitative diary study.

BACKGROUND: Parents have a vital influence on the participation of their child with a physical disability. The aim of this study is to gain insight into parents' own daily actions, challenges, and needs while supporting their child with a physical disability at home, at school, and in the community. An additional objective of this study is to refine the preliminary thematic framework previously identified in a scoping review. METHODS: A qualitative research inquiry was performed based on using a diary over a 7-day period to gather data. To systematically organise data into a structured format, content analysis has been applied using both inductive and deductive reasoning guided by the existing preliminary thematic framework. RESULTS: Analysis of the eligible diaries shows that the actions mentioned by the 47 parents describe several efforts to enhance participation of their children with a physical disability by using, enabling, or changing the social and physical environment, or by supporting their child to perform or engage in meaningful activities. Those parents' actions are primarily a result of challenges caused by restrictions in social and physical environments. Parental responses highlighted, above all, the need for environments designed for all people. Based on the findings a redefined thematic framework is presented. CONCLUSIONS: Parents' actions, challenges, and needs are mainly directed towards the social or/and physical environment. The presented thematic framework can offer practitioners knowledge to support parents. More work is necessary to provide tailored approaches. Paediatric rehabilitation may need to address the importance of the environment on the participation of a child with a physical disability.

Attachment Representation and Sensitivity: The Moderating Role of Posttraumatic Stress Disorder in a Refugee Sample.

It has been hypothesized that adult attachment representations guide caregiving behavior and influence parental sensitivity, and thus affect the child's socio-emotional development. Several studies have shown a link between posttraumatic stress disorder (PTSD) and reduced parental sensitivity, so it is possible that PTSD moderates the relationship between insecure attachment representations and insensitivity. In this study symptoms of PTSD (Harvard Trauma Questionnaire), parental sensitivity (Emotional Availability Scales), and attachment representations (Attachment Script Assessment) were assessed in 53 parents who were asylum seekers or refugees. Results showed that when parents were less able to draw on secure attachment representations, symptoms of PTSD increased the risk of insensitive parenting. These findings suggest that parental sensitivity is affected not just by attachment representations, but by a conjunction of risk factors including symptoms of PTSD and insecure attachment representations. These parents should therefore be supported to establish or confirm secure models of attachment experiences, to facilitate their ability interact sensitively and form a secure relationship with their children.

Efficacy of three therapy approaches in preschool children with cerebral palsy: a randomized controlled trial.

AIM: To examine the efficacy of child-focused, context-focused, and regular care approaches, delivered in a rehabilitation setting by physical or occupational therapists to preschool children with cerebral palsy (CP), in optimizing the child's self-care and mobility capabilities. METHOD: A multicentre randomized controlled trial clustered at therapist level was conducted in 13 rehabilitation centres. It included 68 children with CP (38 males, 30 females; mean age 3y, SD 6mo, range 1y 11mo-4y), classified as Gross Motor Function Classification System levels I to IV, who were already receiving therapy. Children received a child-focused, context-focused, or regular care approach during a 6-month period. Self-care and mobility capabilities were assessed with the Functional Skills Scale of the Pediatric Evaluation of Disability Inventory. RESULTS: The child-focused, context-focused, and regular care approaches all resulted in significant but similar improvements in self-care (regular: reference; child-focused: ß=-0.11, 95% confidence interval [CI] -0.68 to 0.46; context-focused: ß=0.13, CI -0.38 to 0.64) and mobility (regular: reference; child-focused: ß=-0.09, CI=-0.93 to 0.75; and context-focused: ß=0.14, CI -0.65 to 0.94) capabilities. INTERPRETATION: The results suggest that the three therapy approaches were equally efficacious for preschool children with CP. Depending on a child's individual situation each approach can be selected.

Parental PTSD, adverse parenting and child attachment in a refugee sample.

In contrast with traumatic experiences, there is a dearth of studies on the link between trauma symptoms, disconnected (frightened, threatening and dissociative) parenting behavior, extremely insensitive parenting behavior and child attachment. This study extends previous work on the impact of posttraumatic stress disorder (PTSD) on families by studying the unique contribution of disconnected and extremely insensitive parenting behavior on child attachment in a highly traumatized sample of 68 asylum seekers and refugees and their children (18-42 months). The results show that parental symptoms of PTSD are directly related to children's insecure attachment and disorganized attachment. The greatest proportion of the risk could be attributed to factors related to the dyad and not the family. A mediation effect of adverse parenting behavior was not confirmed. On the one hand the results indicate the need for an effective treatment of PTSD symptomatology while on the other hand the results indicate the need for clinical attention to insecure attachment relationships.

Child-Focused and Context-Focused Behaviors of Physical and Occupational Therapists during Treatment of Young Children with Cerebral Palsy.

AIMS: To (1) describe the child- and context-focused behaviors of physical and occupational therapists, and (2) compare the behaviors of therapists in a standard therapy session with those of therapists trained to deliver child- and context-focused services. METHOD: Videos of 49 therapy sessions provided by 36 therapists were analyzed using the intervention domains of the Paediatric Rehabilitation Observational measure of Fidelity (PROF) to examine the therapeutic behaviors of physical and occupational therapists with young children with cerebral palsy (CP) (24 to 48 months) in a Dutch rehabilitation setting. The PROF ratings of 18 standard therapy sessions were compared with the ratings of 16 child- and 15 context-focused therapy sessions. RESULTS: Therapists who provided standard therapy demonstrated a mix of child- and context-focused behaviors. PROF ratings indicated fewer child- and context-focused behaviors during standard therapy sessions compared with sessions where therapists were instructed to use either child- or context-focused behaviors. CONCLUSIONS: A sample of Dutch physical and occupational therapists of young children with CP demonstrated a mix of child- and context-focused therapy behaviors during standard therapy. Further research is recommended on clinical reasoning and the effect of setting to better understand therapists' use of child- and context-focused behaviors during therapy sessions.

Surviving a brain tumor in childhood: impact on family functioning in adolescence.

OBJECTIVE: To investigate family functioning in families with an adolescent survivor of a pediatric brain tumor. We explored whether adolescent, parent, disease and treatment factors, and demographic characteristics predicted family functioning. METHODS: In this cross-sectional study, 45 adolescent survivors of pediatric brain tumors and their parents completed self-report questionnaires on family functioning, and emotional and behavioral problems. Parents completed questionnaires on their own mental health and the burden of treatment. RESULTS: Compared to general population norms, adolescents reported higher levels of cohesion, expressiveness, organization, control, family values and social orientation, and absence of conflict. Parents reported higher levels of social orientation and lower levels of conflict and family values. The only predictor of family functioning was current age of the adolescent; older adolescents reported less family conflict. No relation was found between family functioning and emotional and behavioral problems, disease- or treatment factors, and demographic variables. CONCLUSIONS: In this exploratory study, adolescent survivors of a pediatric brain tumor characterized their families by higher levels of cohesion, expressiveness, organization, control, family values and social orientation, and absence of conflict, which differs from the more normative view held by their parents. A higher adolescent age predicted less family conflict, which may indicate deviant autonomy development in these survivors. Because of limitations of this study, conclusions should be considered provisional; they provide clues for further research in this area.

Participation and social participation: are they distinct concepts?

INTRODUCTION: The concept of participation has been extensively used in health and social care literature since the World Health Organization introduced its description in the International Classification of Functioning, Disability and Health (ICF) in 2001. More recently, the concept of social participation is frequently used in research articles and policy reports. However, in the ICF, no specific definition exists for social participation, and an explanation of differences between the concepts is not available. AIM: The central question in this discussion article is whether participation, as defined by the ICF, and social participation are distinct concepts. This article illustrates the concepts of participation and social participation, presents a critical discussion of their definitions, followed by implications for rehabilitation and possible future directions. DISCUSSION: A clear definition for participation or social participation does not yet exist. Definitions for social participation differ from each other and are not sufficiently distinct from the ICF definition of participation. Although the ICF is regarded an important conceptual framework, it is criticised for not being comprehensive. The relevance of societal involvement of clients is evident for rehabilitation, but the current ICF definition of participation does not sufficiently capture societal involvement. CONCLUSION: Changing the ICF's definition of participation towards social roles would overcome a number of its shortcomings. Societal involvement would then be understood in the light of social roles. Consequently, there would be no need to make a distinction between social participation and participation.

Primary Care Triple P for parents of NICU graduates with behavioral problems: a randomized, clinical trial using observations of parent-child interaction.

BACKGROUND: Preterm-born or asphyxiated term-born children show more emotional and behavioral problems at preschool age than term-born children without a medical condition. It is uncertain whether parenting intervention programs aimed at the general population, are effective in this specific group. In earlier findings from the present trial, Primary Care Triple P was not effective in reducing parent-reported child behavioral problems. However, parenting programs claim to positively change child behavior through enhancement of the parent-child interaction. Therefore, we investigated whether Primary Care Triple P is effective in improving the quality of parent-child interaction and increasing the application of trained parenting skills in parents of preterm-born or asphyxiated term-born preschoolers with behavioral problems. METHODS: For this pragmatic, open randomized clinical trial, participants were recruited from a cohort of infants admitted to the neonatal intensive care units of two Dutch hospitals. Children aged 2-5 years, with a gestational age <32 weeks and/or birth weight <1500 g and children with a gestational age 37-42 weeks and perinatal asphyxia were included. After screening for a t-score ≥60 on the Child Behavior Checklist, children were randomly assigned to Primary Care Triple P (n = 34) or a wait-list control group (n = 33). Trial outcomes were the quality of parent-child interaction and the application of trained parenting skills, both scored from structured observation tasks. RESULTS: There was no effect of the intervention on either of the observational outcome measures at the 6-month trial endpoint. CONCLUSIONS: Primary Care Triple P, is not effective in improving the quality of parent-child interaction nor does it increase the application of trained parenting skills in parents of preterm-born or asphyxiated term-born children with behavioral problems. Further research should focus on personalized care for these parents, with an emphasis on psychological support to reduce stress and promote self-regulation. TRIAL REGISTRATION: Netherlands National Trial Register NTR2179 . Registered 26 January 2010.

Determinants of Developmental Gain in Daily Activities in Young Children with Cerebral Palsy.

ABSTRACT The aim of this study was to examine which child and family characteristics at the child's age of 2 years are determinants of development of self-care and mobility activities over a period of 2 years in young children with cerebral palsy (CP). Longitudinal data of 92 children, representing all levels of the Gross Motor Function Classification System (GMFCS), were analyzed. Children's self-care and mobility activities were assessed with the Functional Skills Scale of the Pediatric Evaluation of Disability Inventory. Development of self-care and mobility activities was related to several child determinants but no family determinants. GMFCS, type of CP, intellectual capacity, and epilepsy were related to the development of self-care and mobility activities, while manual ability and spasticity were related to development of mobility activities. Multivariate analysis indicated that GMFCS and intellectual capacity were the strongest determinants of development of self-care activities, and GMFCS was the strongest determinant of development of mobility activities. The change in self-care and mobility activities was less favorable in severely affected children with severe disability. Knowledge of GMFCS level and intellectual capacity is important in anticipating change over time and goal setting in young children with CP.

Quality of life in SCN1A-related seizure disorders across the lifespan.

This cohort study aims to describe the evolution of disease features and health-related quality of life per life stage in Dravet syndrome and other SCN1A-related non-Dravet seizure disorders which will enable treating physicians to provide tailored care. Health-related quality of life and disease features were assessed cross-sectionally in participants with a SCN1A-related seizure disorder, categorized per age group for Dravet syndrome, and longitudinally over seven years follow-up (2015-2022). Data were collected from questionnaires, medical records, and semi-structured telephonic interviews. Health-related quality of life was measured with the Paediatric Quality of Life Inventory, proxy-reported for participants with Dravet syndrome and for participants with non-Dravet aged younger than 18 years old and self-reported for participants with non-Dravet over 18 years old. Associations between health-related quality of life and disease features were explored with multivariable regression analyses, cross-sectionally in a cohort of 115 patients with Dravet and 48 patients with generalized epilepsy with febrile seizures plus and febrile seizures (non-Dravet) and longitudinally in a cohort of 52 Dravet patients and 13 non-Dravet patients. In the cross-sectional assessment in 2022, health-related quality of life was significantly lower in Dravet syndrome, compared to non-Dravet and normative controls. Health-related quality of life in the School and Psychosocial domain was significantly higher in older Dravet age groups. A higher health-related quality of life was associated with fewer behavioural problems [ß = -1.1; 95% confidence interval (CI), (-1.4 to -0.8)], independent walking (ß = 8.5; 95%CI (4.2-12.8)), compared to the use of a wheelchair), and fewer symptoms of autonomic dysfunction (ß = -2.1, 95%CI (-3.2 to -1.0)). Longitudinally, health-related quality of life was significantly higher seven years later in the course of disease in Dravet participants (Δ8.9 standard deviation (SD) 18.0, P < 0.05), mediated by a lower prevalence of behavioural problems (ß = -1.2, 95%CI (-2.0 to -0.4)), lower seizure frequency (ß = -0.1, 95%CI (-0.2 to -0.0)) and older age (ß = 0.03, 95%CI (0.01-0.04)). In summary, health-related quality of life was significantly higher at older age in Dravet syndrome. This finding may reflect the benefits of an advanced care strategy in recent years and a ceiling of severity of disease symptoms, possibly resulting in an increased wellbeing of parents and patients. The strong association with behavioural problems reinforces the need to incorporate a multidisciplinary approach, tailored to the age-specific needs of this patient group, into standard care.

Brief parenting intervention for parents of NICU graduates: a randomized, clinical trial of Primary Care Triple P.

BACKGROUND: Preterm-born or asphyxiated term-born children who received neonatal intensive care show more emotional and behavioral problems than term-born children without a medical condition. It is uncertain whether regular parenting intervention programs to which the parents of these children are usually referred, are effective in reducing child problem behavior in this specific population. Our objective was to investigate whether a regular, brief parenting intervention, Primary Care Triple P, is effective in decreasing emotional and behavioral problems in preterm-born or asphyxiated term-born preschoolers. METHODS: For this pragmatic, open randomized clinical trial, participants were recruited from a cohort of infants admitted to the neonatal intensive care units (NICU) of two Dutch hospitals. Children born with a gestational age <32 weeks or birth weight <1500 g and children born at a gestational age 37-42 weeks with perinatal asphyxia were included. After screening for a t-score ≥60 on the Child Behavior Checklist (CBCL), children were randomly assigned to Primary Care Triple P (n = 34) or a wait-list control group (n = 33). The primary outcome was child emotional and behavioral problems reported by parents on the CBCL, 6 months after the start of the trial. RESULTS: There was no effect of the intervention on the CBCL at the trial endpoint (t64 = 0.54, P = .30). On secondary measurements of child problem behavior, parenting style, parenting stress, and parent perceived child vulnerability, groups either did not differ significantly or the intervention group showed more problems. In both the intervention and control group there was a significant decrease in emotional and behavioral problems during the trial. CONCLUSIONS: Primary Care Triple P, a brief parenting intervention, is not effective in reducing child emotional and behavioral problems in preterm-born children or term-born children with perinatal asphyxia. TRIAL REGISTRATION: Netherlands National Trial Register (NTR): NTR2179.