Effectiveness of Chloral Hydrate on Brain MRI in Children with Developmental Delay/Intellectual Disability Comparing with Normal Intelligence: Single Tertiary Center Experience

Neurodiagnostic investigation requirements are expanding for diagnostic and therapeutic purposes in children, especially in those with developmental delay/intellectual disability (DD/ID). Thus, determination of optimal sedatives to achieve successful sedation and immobility without further neurological compromise is important in children with DD/ID. The purpose of this study is to assess the effectiveness and adverse reactions of chloral hydrate (CH) for brain magnetic resonance imaging (B-MRI) in children with DD/ID compared to those with normal intelligence (NI). We performed a retrospective chart review of children aged from 1 day to 12 years who required elective sedation using CH for B-MRI. About 730 cases (415 with DD/ID and 315 with NI) of CH sedation were conducted for B-MRI. Children with DD/ID showed a higher failure rate (22%) than did those with NI (6%); additional CH and prolonged sedation time were required. There was no difference in incidence of adverse reactions between DD/ID and NI groups (p = 0.338). Older or heavier children with DD/ID (p = 0.036 and p = 0.013, respectively), as well as those diagnosed with epilepsy or neuropsychiatric disorders showed higher risk of sedation failure (p < 0.001 for each). In conclusion, CH was a suboptimal sedative drug for children with DD/ID compared with those with NI. Other alternative or supplementary sedatives should be taken into consideration especially for those vulnerable groups.

Comparative Efficacy of Levetiracetam for Epilepsy in School-Aged Children with Intellectual Disability and Normal Intelligence

Choosing optimal anti-seizure medication (ASM) is very important in pediatric patients with epilepsy who attend school, especially children with an intellectual disability (ID). Levetiracetam (LEV) has proven to be an effective, safe, generally well-tolerated, broad-spectrum ASM in children. In the context of increasing use of LEV in school-aged children with epilepsy and ID, we evaluate relevant clinical data, including efficacy, safety, and tolerability in children with epilepsy and an intellectual disability (ID) or normal intelligence (NI). We performed a retrospective chart review of children and included 298 pediatric patients with epilepsy who were treated with LEV with NI (147) and ID (151). After 6 months, 96% of NI and 83% of ID subjects had a seizure reduction rate greater than 50% (p = 0.031). The tolerability of LEV was generally good, with 75% retention rates at 2 years in both groups and only minor side effects (under 15%). The retention rates of patients with NI and ID were 76% and 74%, respectively (p = 0.597). Thus, LEV showed considerable efficacy with minimal side effects and high retention rates and is an easily maintained and safe treatment option for pediatric epilepsy with ID. However, better-designed research studies are needed to clearly elucidate the efficacy and safety of LEV in children with epilepsy and ID.

Pola Asuh Anak Cerdas Istimewa dalam Islam

This study aims to determine how Islamic parenting is applied to special intelligent and gifted children. A special intelligent child is a child who has intelligence above the average of his peers. They are children who have high independence, have self-taught behavior, cannot be detected by other people and can create ideas spontaneously. Special intelligent children have intelligence above 130 (Waschler scale), they have high-order thinking patterns and cannot be equated with their peers who only have normal intelligence. The pattern of education and parenting applied by parents to their children can use various methods that have been taught by the Prophet in Islamic religious teachings. These methods include modeling methods and noble ethics, situational and conditional methods, questioning methods, analogical methods, and dialogical interaction methods. This research was conducted to provide information about proper parenting in Islam through the methods taught by the Prophet.

Evaluation of Psychometric Indicators Persian Version of Behavior Assessment System for Children (BASC-3) in Girls And Boys with Mild Intellectually Disabled and Normal Intelligence (PRS-A & TRS-A) Form

Objective: The purpose of the present study was evaluation of psychometric indicators Persian version of Behavior Assessment System for Children (BASC-3) in girls and boys with mild intellectually disabled and normal intelligence in the city of Yazd. Methods: The present study was a cross-sectional study. This study was performed on girls and boys with mild intellectually disabled and normal intelligence in 2016-2017. The research sample consisted of 438 students who were selected by using cluster sampling method. The data collection tools included (BASC-3), Parental form (PRS-A) and teacher form (TRS-A). Data analysis were performed in SPSS v.24 using descriptive and inferential statistics. Results: The results obtained using factor analysis, correlation coefficient of subscales with total score, test-retest and Cronbachchr('39')s alpha showed that (BASC-3) system has appropriate validity and reliability for both groups, and PRS-A & TRS-A form can be used for both groups with mild intellectually disabled and normal intelligence. Conclusion: According to the results of the present study, it can be said that (BASC-3), Parental form (PRS-A) and teacher form (TRS-A), have appropriate psychometric properties and can be used with confidence to evaluate the behavioral and adaptive problems of adolescents and young people with mild intellectually disabled and normal intelligence in Iran.

Answer Selection Process and Error Detection when Solving Simple Cognitive Tasks

The study is aimed at examining the process of erroneous and correct answers when solving simple cognitive tasks of the same type in the group of adolescents with normal intelligence and those with mild mental deficiency. To study the nature of making errors the author studied factors influencing the process of answer selection, such as conditions of stimulus presentation, stimuli categorical congruence, and unconscious error detection markers of various kinds. The study revealed that if adolescents with normal intelligence make mistakes they do it trying to do the task quickly, while adolescents with mild mental deficiency make mistakes because of the task difficulty and demonstrate a low level of mindfulness of making a mistake, and slow-up adjustment process after a correct answer. Depending on whether there being or not being mental deficiency, certain differences in the process of error detection and the influence of conditions of stimulus presentation were detected.

Turner Syndrome with Ring X Chromosome: Do They Have a Distinct Phenotype?

In contrast to classic Turner syndrome, Turner patients with ring X chromosome are associated with distinct dysmorphism and are likely to be mentally impaired. Four Turner patients with ring X chromosome were examined for phenotypic features of Turner syndrome and additional dysmorphism. Both patients 1 and 2 are twins with normal intelligence whereas patients 3 and 4 have mental impairment. With the exception of patient 4, the other three patients only have few Turner characteristics. None of the patients have the distinctive dysmorphism previously reported in Turner syndrome with ring X chromosome. Both twins developed spontaneous puberty. Patients 3 and 4 however had no spontaneous puberty. We postulate that this variation may be related to the ring size, the proportion of 45,X and ring X chromosome in cell lines of various body tissues as well as the ability of these rings to be inactivated as a result of lyonisation.

Autism Traits Predict Self-reported Executive Functioning Deficits in Everyday Life and an Aversion to Exercise

Are Autism Quotient (AQ) scores related to executive functioning (EF)? We sampled 200 students of normal intelligence and examined the relationship between AQ scores and: (a) 5 self-ratings of EF, (b) 5 performance-based measures of EF, and (c) 5 types of activities or experiences that are assumed to recruit EF and sometimes enhance EF. Our findings reveal that as AQ scores increase, self-rated EF ability decreases. AQ scores and self-reported EF measures do not correlate with objective EF task performance. Furthermore, AQ scores were shown to be negatively associated with many specific types of physical activity. As AQ scores increase, individuals report fewer positive reasons for exercise and more rationalizations for not engaging in more exercise.

Normal early development in siblings with novel compound heterozygous variants in ASPM

Autosomal recessive primary microcephaly 5 (MCPH5) is caused by pathogenic variants in ASPM. Using whole-exome sequencing, we diagnosed two siblings with MCPH5. A known pathogenic variant (NM_018136.4: c.9697C > T, p.(Arg3233*)) and a novel pathogenic variant (c.1402_1406del, p.(Asn468Serfs*2)) of ASPM were identified in affected siblings with normal intelligence. Their pathogenic variants were not located in the critical regions of ASPM, but the relationship between the genotypes and their normal intelligence was unclear.