Cochlear implantation outcomes in children with multiple disabilities: a topic that's worth revisiting.

OBJECTIVES: To determine the benefits of cochlear implantation in hearing loss children with multiple disabilities (MD) in terms of auditory outcomes, speech performance, and their quality of life. METHODS: This was a cross sectional study from January 2019 to December 2020 in which thirty-one children with hearing loss and multiple disabilities were evaluated. Their improvement in auditory and speech performances were assessed using Categories of Auditory Performance version II (CAP-II) and the Speech Intelligibility Rating (SIR) scales. The assessment was done at 6-month intervals, with the baseline evaluation done at least six months after activation of the implant. Parents were asked to fill the Parents Evaluation of Aural/Oral Performance of Children (PEACH) diary and Perceived Benefit Questionnaire (PBQ) to evaluate the child's quality of life. RESULTS: All 31 children have Global Developmental Delay (GDD), with 11 having an additional disability. Both mean CAP-II and SIR scores showed significant improvement with increased hearing age (p < 0.05) after 6-month intervals. In addition, 20 out of 31 children (64.5%) have achieved verbal communication after implantation. The mean PEACH score in quiet was significantly better than in noise (p = 0.007) and improved with the increased of hearing age. The majority of parents (96%‒100%) perceived a cochlear implant as beneficial to their child in terms of auditory response, awareness, interaction, communication, and speech development. CONCLUSIONS: Cochlear implantation had shown benefits in children with multiple disabilities. Outcome measures should not only focus on auditory and speech performances but the improvement in quality of life. Hence, individualized each case with realistic expectation from families must be emphasized in this group of children. LEVEL OF EVIDENCE: Level 3.

Estimating the Risk of Attention Deficit Hyperactivity Disorder (ADHD) in Parents of Children with ADHD and the Association with Their Children's Disease Severity and Adherence to Medication.

BACKGROUND: Attention deficit hyperactivity disorder (ADHD) is characterised by inattentiveness, hyperactivity, and impulsivity. Up to half of the affected children have a parent with ADHD. In this study, the risk of ADHD among parents of ADHD children was estimated. The associations between parental ADHD and child ADHD severity and medication adherence were determined. METHODOLOGY: Parents of children and adolescents diagnosed with ADHD attending the University Kebangsaan Malaysia Medical Centre (UKMMC) were recruited between June to August 2022 and the administered Conners' Adult ADHD Rating Scale (CAARS) self-report short form, Vanderbilt ADHD Parent Rating Scale (performance section), and Medication Adherence Report Scale (MARS). RESULTS: Forty-five children with ADHD were recruited and 15 out of 45 (33%) parents were detected to have ADHD. ADHD severity was worse in children with ADHD parents for total severity (mean of 34.67 vs. 29.13, p = 0.047) and difficult behaviours at home (mean of 7.87 vs. 6.27, p = 0.036). The children's academic performance and behavioural challenges at home and school were positively correlated with the parental ADHD scores for 'inattention' and 'problems with self-care' subscales. CONCLUSIONS: A total of 33% of ADHD children had parents with ADHD. ADHD children with ADHD parents were more likely to have behavioural problems at home and more severe ADHD. However, no statistical significance was noted with medication adherence.

Motor Development in Children With Autism Spectrum Disorder.

Objective: This study was conducted to determine the gross and fine motor profiles of children with autism spectrum disorder compared to typically developing children. Additionally, we also assessed if the motor delay was more pronounced with increasing age. Method: This was a retrospective study involving children aged 12-60 months of age comparing motor development in children with autism spectrum disorder with typically developing children. Their developmental profile was assessed using Schedule of Growing Skills II. Descriptive statistics was used to analyse the developmental profile between the groups. Results: ASD children had significant gross motor (6.7%) and fine motor delay (38.5%) compared to typically developing children, who did not show any delay. The motor delay in ASD children was more prominent in older children. Conclusion: It is important to assess motor development in ASD children as there is significant motor delay in these children compared to typically developing children, and the delay becomes more prominent with age. Early detection of motor delay could allow provision of early intervention services to optimize developmental outcomes.

Association between Viral Infections and Risk of Autistic Disorder: An Overview.

Autism spectrum disorder (ASD) is a neurodevelopmental condition of the central nervous system (CNS) that presents with severe communication problems, impairment of social interactions, and stereotypic behaviours. Emerging studies indicate possible associations between viral infections and neurodegenerative and neurobehavioural conditions including autism. Viral infection during critical periods of early in utero neurodevelopment may lead to increased risk of autism in the offspring. This review is aimed at highlighting the association between viral infections, including viruses similar to COVID-19, and the aetiology of autism. A literature search was conducted using Pubmed, Ovid/Medline, and Google Scholar database. Relevant search terms included "rubella and autism", "cytomegalovirus and autism", "influenza virus and autism", "Zika virus and autism", "COVID-19 and autism". Based on the search terms, a total of 141 articles were obtained and studies on infants or children with congenital or perinatal viral infection and autistic behaviour were evaluated. The possible mechanisms by which viral infections could lead to autism include direct teratogenic effects and indirect effects of inflammation or maternal immune activation on the developing brain. Brain imaging studies have shown that the ensuing immune response from these viral infections could lead to disruption of the development of brain regions and structures. Hence, long-term follow up is necessary for infants whose mothers report an inflammatory event due to viral infection at any time during pregnancy to monitor for signs of autism. Research into the role of viral infection in the development of ASD may be one avenue of improving ASD outcomes in the future. Early screening and diagnosis to detect, and maybe even prevent ASD are essential to reduce the burden of this condition.

Missed Appointments at a Child Development Centre and Barriers to Access Special Needs Services for Children in Klang Valley, Malaysia: A Mixed Methods Study.

Attending appointments is vital for children with special needs, as such appointments involve long-term interdisciplinary care to ensure continuity of care and improve health and well-being. This study was performed to determine the prevalence of missed clinic appointments and identify the factors among those who have ever missed appointments and barriers of access to children's special needs services at the Child Development Centre (CDC) at the Universiti Kebangsaan Malaysia Medical Centre (UKMMC). Moreover, suggestions for improvement from the caregivers' perspectives were explored. This is an explanatory sequential mixed methods study among caregivers of children with developmental disabilities aged up to 17 years old. Of 197 caregivers, 62 (31.5%) had missed clinic appointments. Forgetfulness was the most frequently cited reason. The bi-variable analysis showed significant differences in missed appointment rates by gender of caregivers and duration of follow-up. The final logistic regression model demonstrated that, when combined with the effect of being a male caregiver as an independent variable, follow-up duration of more than 6 years increased 2.67 times the risk of missing an appointment. Caregivers' perceived barriers were transportation, caregiver, child and healthcare services factors. Policies and strategic plans should be focused on key findings from these factors to improve appointment adherence and accessibility to services for children with special needs.

Update on Atypicalities of Central Nervous System in Autism Spectrum Disorder.

Autism spectrum disorder (ASD) is a heterogeneous, behaviorally defined, neurodevelopmental disorder that has been modeled as a brain-based disease. The behavioral and cognitive features of ASD are associated with pervasive atypicalities in the central nervous system (CNS). To date, the exact mechanisms underlying the pathophysiology of ASD still remain unknown and there is currently no cure or effective treatment for this disorder. Many publications implicated the association of ASD with inflammation, immune dysregulation, neurotransmission dysfunction, mitochondrial impairment and cell signaling dysregulation. This review attempts to highlight evidence of the major pathophysiology of ASD including abnormalities in the brain structure and function, neuroglial activation and neuroinflammation, glutamatergic neurotransmission, mitochondrial dysfunction and mechanistic target of rapamycin (mTOR) signaling pathway dysregulation. Molecular and cellular factors that contributed to the pathogenesis of ASD and how they may affect the development and function of CNS are compiled in this review. However, findings of published studies have been complicated by the fact that autism is a very heterogeneous disorder; hence, we addressed the limitations that led to discrepancies in the reported findings. This review emphasizes the need for future studies to control study variables such as sample size, gender, age range and intelligence quotient (IQ), all of which that could affect the study measurements. Neuroinflammation or immune dysregulation, microglial activation, genetically linked neurotransmission, mitochondrial dysfunctions and mTOR signaling pathway could be the primary targets for treating and preventing ASD. Further research is required to better understand the molecular causes and how they may contribute to the pathophysiology of ASD.

Sleep Disturbances in Children With Autism Spectrum Disorder at a Malaysian Tertiary Hospital.

Introduction: Children with autism spectrum disorder (ASD) have a variety of co-morbid medical problems, including sleep disturbances. Prevalence of sleep disorders has been reported to be higher in this group as compared to the general population. Identifying sleep problems in children with ASD may help increase awareness and improve the overall quality of care for them. The aim of this study was to determine the prevalence of sleep problems and associated factors in a group of Malaysian children aged 6-16 years, with ASD. Method: This is a cross-sectional study at the Child Development Centre of UKM Medical Centre (UKM MC) on ASD children aged 6-16 years. Demographic data was obtained and the Sleep Disturbances Scale for Children (SDSC) questionnaire was completed by the main caregiver. Logistic regression analysis was used to determine factors related to higher total SDSC scores. Results: A total of 128 patients were recruited (111 boys) with a median age of 8 years 3 months (IQR: 2 years 10 months). Forty-seven (36.7%) of them obtained total SDSC scores in the pathological range with 19 (14.8%) scoring high for overall disturbances and 28 (21.9%) for at least one subtype of sleep disorders: 25 (19.5%) DIMS, 18 (14.1%) SBD, 10 (7.8%) DOES, 5 (3.9%) DOA, 6 (4.7%) SWTD, and 3 (2.3%) SHY. More than half of the children (57.8%) were reported to have sufficient sleep duration of 8-11 h, but longer sleep latency of at least 15 min (82.8%). Half of the ASD children also had co-morbidities in which one-third (34.4%) had attention-deficit hyperactivity disorder (ADHD). Using logistic regression analysis, four factors were significantly associated with higher total SDSC scores; female gender (p = 0.016), older age group (11-16 years old) (p = 0.039), shorter sleep length (p = 0.043), and longer sleep latency (p < 0.001). Conclusion: The prevalence of sleep disturbances is high among Malaysian children with ASD, especially DIMS. Female gender, older age group, shorter sleep length, and longer sleep latency were found to be associated with the sleep disturbances. Evaluation of sleep problems should form part of the comprehensive care of children with ASD.

Prevalence of Overweight and Obesity Among Children and Adolescents With Autism Spectrum Disorder and Associated Risk Factors.

Introduction: Prevalence of obesity in Autism Spectrum Disorder (ASD) has been reported to be higher than in the general population. Determining prevalence may help increase awareness of obesity in ASD and potentially lead to initiatives to reduce obesity. In order to understand obesity in ASD children, common risk factors were assessed including physical activity, feeding problems and sleep disturbances. Methods: This is a cross-sectional study performed at the Child Development Center at Universiti Kebangsaan Malaysia Medical Center on 151 ASD children aged 2-18 years. Anthropometric and demographic information were obtained and parents completed three questionnaires; Children Sleep Habits Questionnaire (CSHQ), Physical Activity for Older Children Questionnaire (PAQ-C) and Brief Autism Mealtime Behavior Questionnaire (BAMBI). Results: For ASD children in our sample, the prevalence of overweight (BMI ≥85th to <95th percentiles) was 11.3% and the prevalence of obesity (BMI ≥95th percentile) was 21.9%. The overweight/obese ASD children's median age was higher at 8.5 years (IQR 5.81-10.13) compared to the normal/underweight group of 6.33 years (IQR 4.75-7.7) with a p-value of 0.001. The two groups also differed significantly for maternal BMI and paternal age. The median maternal BMI in the overweight/obese group was 26.05 (IQR 23.35-32.25), statistically significantly higher (p = 0.003) than in the non-overweight/obese group, 24.7 (IQR 21-27.9). The median paternal age of 40 years (IQR 37-44) was statistically significantly higher (p = 0.039) in the overweight/obese group, compared to the median paternal age in the non-overweight/obese group of 38 (IQR 35-42). The male overweight/obese children had median PAQ-C score of 2.44 (IQR 2.00-3.00) vs. 2.89 (IQR 2.35-3.53) in the counterpart group with a p-value of 0.01. Using the multiple linear regression stepwise method, three predictors associated with BMI percentiles reached a statistical level of significance; PAQ-C score in males (p < 0.001), the BAMBI domains of Food Refusal (p = 0.001) and Limited Variety of Food (p = 0.001). Conclusions: The prevalence of obesity and overweight is high among Malaysian ASD children and adolescents. Older child age, high maternal BMI, older paternal age, low physical activity, low likelihood of food refusal and high likelihood of food selectivity were found to be risk factors for high BMI in these children.

Neonatal hypoxic encephalopathy: Correlation between post-cooling brain MRI findings and 2 years neurodevelopmental outcome.

OBJECTIVE: This study aims to evaluate the magnetic resonance imaging (MRI) brain patterns among hypoxic-ischemic encephalopathy (HIE) babies who underwent post-cooling MRI brain as well as to correlate the post-cooling brain scoring with patient's neurodevelopmental outcome at 2 years. SUBJECTS AND METHODS: It was a retrospective cross sectional study carried out at a tertiary university hospital. Record of patients diagnosed with neonatal HIE from 2007 until 2016 who completed 72 h of cooling therapy and had MRI brain within 2 weeks of life were included in this study. A new scoring system by Trivedi et al. that emphasizes on subcortical deep gray matter and posterior limb internal capsule injury were utilized upon MRI assessment, using TW, T2W, and diffusion-weighted imaging (DWI) sequences. Cumulative MRI brain score was obtained and graded as none, mild, moderate, and severe brain injury. The MRI brain scoring was then correlated with patient's 2 years neurodevelopmental outcome using Fisher's Exact Test. RESULTS: A total of 23 patients were eligible of which 19 term neonates were included. 13% of these neonates (n = 3) had mild MRI brain injury grading with 52.2% (n = 12) moderate and 34.8% (n = 8) severe. There was no significant correlation seen between MRI brain grading and developmental outcome at 2 years old (P > 0.05). CONCLUSION: There was no significant correlation between neonatal MRI brain injury grading and 2 years neurodevelopmental outcome. Nevertheless, the new MRI brain scoring by Trivedi et al. is reproducible and comprehensive as it involves various important brain structures, assessed from different MRI sequences.