Behavioral Problems
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2021 ◽  
Vol 15 ◽  
Huiting Zhang ◽  
Liu Yang ◽  
Jing Duan ◽  
Qi Zeng ◽  
Li Chen ◽  

Objective: We aimed to explore the associated clinical phenotype and the natural history of patients with SYNGAP1 gene variations during early childhood and to identify their genotype–phenotype correlations.Methods: This study used a cohort of 13 patients with epilepsy and developmental disorder due to SYNGAP1 mutations, namely, 7 patients from Shenzhen Children’s Hospital between September 2014 and January 2020 and 6 patients from previously published studies. Their clinical data were studied.Results: A total of 13 children with SYNGAP1 gene variants (eight boys and five girls) were identified. The age of disease onset was in infancy. Mutations were located between exons 8 and 15; most were frameshift or truncated mutations. Four mutation sites (c.924G > A, c.1532-2_1532del, c.1747_1755dup, and c.1735_1738del) had not been reported before. All patients had global developmental delay within the first year of life, and intellectual impairment became gradually apparent. Some of them developed behavioral problems. The developmental delay occurred before the onset of seizures. All seven patients in our cohort presented with epilepsy; myoclonic seizures, absence seizures, and epileptic spasms were the most common seizure types. Abnormal electroencephalograms were identified from five patients before the onset of their seizures. All patients suffered from drug-resistance seizures. However, comorbidities such as behavioral problems were less frequently observed.Conclusion: The most common age of disease onset in SYNGAP1 gene mutations is in infancy, while neurodevelopmental delay and epilepsy are the major phenotypes. They have a higher percentage of drug-resistant epilepsy and epileptic spasms than those in previous reports. We should give attention to the patients with abnormal EEGs without seizures and think about the suitable time of the anti-seizure medications for them. We have not found the genotype–phenotype correlation.Trial registration: Chinese Clinical Trial Registry, Registration number: ChiCTR2100049289 (

2021 ◽  
Vol Publish Ahead of Print ◽  
Lu-Chi Hsiao ◽  
Shuenn-Nan Chiu ◽  
Ling-Yin Chang ◽  
Chia-Ching Wang ◽  
Wen-Chin Weng ◽  

2021 ◽  
Vol 12 ◽  
Konstantin L. Makridis ◽  
Sebastian Triller ◽  
Deniz A. Atalay ◽  
Christine Prager ◽  
Christian E. Elger ◽  

Background: Hemispherotomy is an epilepsy surgery procedure applied to cure particularly pharmacorefractory lesional epilepsy due to unihemispheric pathologies. Such a disconnection of an entire hemisphere is followed by reorganizational processes.Methods: We describe an acute aggravation of behavioral problems following a hemispherotomy in a patient treated with valproic acid, which subsided once valproate was discontinued.Results: A 9-year-old boy with drug-resistant epilepsy caused by the residua of a perinatal stroke treated for several years with valproic acid and lamotrigine underwent hemispherotomy. Shortly after surgery, minimal preoperative behavioral problems intensified dramatically, and aggression occurred as a new symptom. Assuming a correlation between valproate treatment and the postoperative altered neuronal network, we tapered off valproate. The behavioral problems decreased in intensity with the reduction of valproate dose and disappeared after drug discontinuation.Conclusion: We describe severe behavioral problems after hemispherotomy that subsided when valproate was tapered off. While we cannot rule out a spontaneous correction of a post-hemispherotomy network dysregulation, our report raises awareness to possible altered effects of the anticonvulsant valproic acid parallel to reorganizational processes after hemispherotomy.

Royce Anders ◽  
Florian Lecuelle ◽  
Clément Perrin ◽  
Swann Ruyter ◽  
Patricia Franco ◽  

It is still debated whether lockdown conditions in response to the coronavirus disease 2019 (COVID-19) health crisis seriously affected children’s sleep. For young children, some studies identified more insomnia, while others only transient disturbances, or even no effect. Based on the premise of mother–child synchrony, a well-known dynamic established in child development research, we hypothesized that principally, the children whose mothers perceived the lockdown as stressful and/or responded maladaptively, suffered sleep disturbances. The main objective of this study was to identify the family profiles, variables, and lockdown responses most linked to insomnia in young children. The sample consisted of 165 mothers, French vs. Swiss origin (accounting for different lockdown severities), of children 6 months to 5 years old. Validated sleep, stress, and behavior scales were used. Multiple regression, age-matched clustering, and structural equation modeling analyses provided evidence that insomnia in young children is indeed strongly linked to the mother’s reaction to the pandemic and lockdown. Specifically, reactions such as COVID-19 fear/anxiety and obsessive COVID-19 information seeking coincide with heightened vigilance, cascading into reduced child social contact, outings, and increased screen viewing, ultimately culminating in child insomnia and behavioral problems. Mother education level and child day care quality (e.g., home-schooling) were also identified as strong insomnia predictors.

2021 ◽  
Vol 15 ◽  
Francesca Felicia Operto ◽  
Grazia Maria Giovanna Pastorino ◽  
Chiara Scuoppo ◽  
Chiara Padovano ◽  
Valentina Vivenzio ◽  

Background: The aim of our study was to compare adaptive skills, emotional/behavioral problems, and parental stress among children with different severity levels of Autism Spectrum Disorder (ASD) symptoms.Methods: This study included a sample of 88 subjects with ASD (mean age = 6.00 ± 2.70). All subjects underwent standardized neuropsychological tests for the assessment of symptoms of the autism spectrum (Autism Diagnostic Observation Schedule-Second Edition), adaptive level (The Vineland Adaptive Behavior Scales, Survey Interview, 2nd edition), behavioral and emotional problems (Child Behavior CheckList CBCL), and parental stress (Parental Stress Index Short Form-PSI-SF). Non-parametric statistical methods (Kruskal-Wallis test and Mann-Whitney U-test for post hoc analysis) and linear regression analysis were used in this study.Results:Children who had higher severity levels of ASD symptoms had less adaptive functioning; younger children showed more severe symptoms of ASD; older children had better communication skills. The presence of greater adaptive difficulties was related to a greater presence of internalizing problems. An increase in parental stress levels was related to an higher severity of ASD symptoms, fewer adaptive skills, and a greater presence of internalizing and externalizing problems.Conclusion: This study suggests that the adaptive behavior should be considered in order to planning a habilitation intervention in children with autism. It is also important to monitor emotional/behavioral problems and parental stress levels in order to provide parenting support and improve the family quality of life.

2021 ◽  
Vol In Press (In Press) ◽  
Fatemeh Mirzaei ◽  
Fatemeh Cheraghi ◽  
Salman Khazaei ◽  
Mohammad Rezaei ◽  
Fatemeh Mohammadi

Background: The disability to recognize emotional and mental states in others’ facial expressions is acknowledged as one of the major developmental-behavioral problems in children with autism. Therefore, it is necessary to study the effect of facial emotion recognition training on the interaction skills of children with autism. Objectives: The aim of this study was to investigate the effect of emotional facial recognition on the mother-child relationship in children with autism. Methods: The present clinical trial was conducted based on a pre-test post-test design. Children aged 6-12 years with high- or moderate-functioning autism were assigned to two groups using the random block sampling method. Children in the intervention group were trained in 10 45-min sessions each week for 10 weeks using 40 images related to four situations of happiness, sadness, anger, and fear. The mother-child relationship questionnaire was completed one day before and one day after the intervention in both groups by the research assistant. Data were analyzed with SPSS software version 22. Results: Emotional state training improved the mother-child relationship in the intervention group. There was also a statistically significant difference between the total scores of the mother-child relationship in the intervention group and the control group (p

Yui Yamaoka ◽  
Aya Isumi ◽  
Satomi Doi ◽  
Manami Ochi ◽  
Takeo Fujiwara

The differential effects of low income and material deprivation—in particular, deprivation related to child educational needs—have not been well examined. This study aimed to examine the effects of low income and life-related and child-related deprivation on child behavioral problems. This study used data from first-grade students who participated in the Adachi Child Health Impact of Living Difficulty (A-CHILD) study in 2015, 2017, and 2019 (N = 12,367) in Japan. Material deprivation was divided into life-related deprivation (i.e., lack of items for a living) and child-related deprivation (i.e., lack of children’s books, etc.), and low income was assessed via annual household income. We assessed child behavioral problems and prosocial behavior using the Strengths and Difficulties Questionnaire. One in ten children belonged to low-income families, 15.4% of children experienced life-related deprivation, and 5.4% of children experienced child-related deprivation. While life- and child-related deprivation had significant adverse effects on behavioral problems, they had no association with prosocial behavior. The effects of low income were mediated by parental psychological distress (45.0% of the total effect) and the number of consulting sources (20.8%) on behavioral problems. The effects of life-related and child-related deprivation were mediated by parental psychological distress (29.2–35.0%) and the number of consulting sources (6.4–6.9%) on behavioral problems. Life-related and child-related deprivation, but not low income, are important for child mental health.

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