Characteristics of Opsoclonus-Myoclonus Syndrome in Patients of the Largest Pediatric Hospital in Latin America.

BACKGROUND: Opsoclonus-myoclonus syndrome (OMS) is a rare neuroinflammatory disorder characterized by ataxia, opsoclonus, and myoclonus. Clinical diagnosis of OMS has been challenging; therefore, we sought to determine the clinical and treatment profiles of patients with OMS at the largest pediatric hospital in Latin America. METHODS: We analyzed the data of patients diagnosed with OMS between 2010 and 2020 at Pequeno Principe Hospital (Brazil) to determine the corresponding clinical profile more accurately. RESULTS: Of the approximately 50,000 visitors to our pediatric neurology department from 2010 to 2020, 10 patients with OMS were observed. Five nontumor cases included three parainfectious and two idiopathic cases. The median time from symptom onset to diagnosis was 34 days. All patients with diagnostic OMS criteria in the idiopathic, nontumor group underwent whole-exome sequencing, with potentially pathogenic mutations identified in two cases. Nine patients were treated with methylprednisolone pulse, followed by oral steroids; eight received one or more intravenous immunoglobulin treatments; and six received azathioprine and cyclophosphamide. Complete symptomatic recovery was observed in only one patient. CONCLUSIONS: OMS diagnosis remains challenging. Diagnostic suspicion is necessary to improve the management of these patients and allow early immunosuppressive treatment. Paraneoplastic etiology is the most prevalent. In idiopathic patients who do not respond to immunosuppressive treatment, tests, such as whole-exome sequencing, may reveal a differential diagnosis. Genetic alterations that increase the risk of tumors may be an important clue to the pathophysiology of OMS.

Family Functioning and Emotional Aspects of Children with Autism Spectrum Disorder in Southern Brazil.

Autism spectrum disorder (ASD) affects children and their families. We investigated the family environment of children with ASD in Brazil. Twenty-one families participated. Outcome measures administered to parents included the Strengths and Difficulties Questionnaire, Questionnaire on Resources and Stress-Short Form, and the Family Environment Scale. All families reported a number of difficulties, including social interactions and peer relationships, stress levels, and communication difficulties. Families also reported great concern for their child's future. Brazil is a developing country, yet there are few national resources and studies related to ASD. The challenges continue from diagnosis through treatment. Our results emphasize the need to understand the family environment of children with autism and their parents' apprehensions to develop effective coping programs.

Autism Spectrum Disorder Diagnoses: A Comparison of Countries with Different Income Levels.

Purpose: This study aimed to assess whether high-income countries have a lower mean age at the time of diagnosis of autism spectrum disorder (ASD) than low- and middle-income countries. Method: We reviewed studies related to ASD diagnoses and the time of first concerns in low-, middle-, and high-income countries, published in PubMed, SciELO, Lilacs, and ScienceDirect. Thirty articles were included: 13 from low- and middle-income countries and 17 from high-income countries. Results: The average delay between initial concerns and diagnosis was 32.33 months, with initial concerns averaging 23.64 months and diagnosis at 55.97 months. No statistical differences were found between countries with low-, middle-, and high-income. Conclusions: This review found a considerable delay in ASD diagnosis despite an early presence of recognized signs and symptoms. It highlights the urgent need for standardized tools for early ASD diagnosis.

Potential Risk Factors for Autism in Children Requiring Neonatal Intensive Care Unit.

BACKGROUND: The etiology of autism spectrum disorder (ASD) is complex and involves the interplay of genetic and environmental factors. AIM: We sought to identify potential prenatal, perinatal, and neonatal risk factors for ASD in a unique population of children who had perinatal complications and required care in a neonatal intensive care unit (NICU). METHODS: This prospective cohort study included 73 patients discharged from a NICU who received long-term follow-up at the largest children's hospital in Brazil. Potential risk factors were compared between 44 children with a diagnosis of ASD and 29 children without using the Mann-Whitney U test. Proportions were analyzed using the chi-square test. Simple and multiple logistic regression tests were performed. RESULTS: Of 38 factors analyzed, the following 7 were associated with ASD: family history of neuropsychiatric disorders (p = 0.049); maternal psychological distress during pregnancy (p = 0.007); ≥ 26 days in the NICU (p = 0.001); feeding tube for ≥ 15 days (p = 0.014); retinopathy of prematurity (p = 0.022); use of three or more antibiotics (p = 0.008); and co-sleeping until up to 2 years of age (p = 0.004). CONCLUSION: This study found associations between specific risk factors during critical neurodevelopmental periods and a subsequent diagnosis of ASD. Knowledge of the etiologic factors that may influence the development for ASD is paramount for the development of intervention strategies and improvement of prognoses.

Association Of Cigarette Smoking With Anxiety, Depression, And Suicidal Ideation Among Brazilian Adolescents.

BACKGROUND: Several studies worldwide have pointed to depression and anxiety symptoms as being related to adolescent smoking. The aim of this study was to investigate, the potential link of cigarette smoking with depression, anxiety, and suicidal ideation and the influence of gender on these relationships in Brazilian adolescents. METHODS: Associations of smoking with Children Depressive Inventory version 2 (CDI2) scores, Hamilton Anxiety Scale (HAM-A) scores, and poor school performance (i.e., grade retention) were examined in 988 Brazilian students (age range, 11-17 years) enrolled in 82 public and private schools. Logistic regression modeling was employed and the resultant odds ratios (ORs) are reported with 95% CIs. RESULTS: Of 988 participants, 240 (24.3%) were smokers. Mean (±standard error) HAM-A scores were higher for smokers (21.1 ± 9.7) than nonsmokers (15.4 ± 8.6; p < 0.0001). Relative to nonsmokers, smokers had higher total CDI2 scores (p = 0.033), and higher scores for the CDI2 domains of Emotional Problems (p = 0.023), Negative Self-esteem (p < 0.001), and Functional Problems (p = 0.046). Suicidal ideation was common among smokers with depressive symptoms (54.2%). Smoking was associated with being held back three grades (p < 0.001). Female smokers were more likely to report suicidal ideation than male smokers (p = 0.020). Logistic regression modeling revealed significant associations of suicidal ideation with being female (OR, 1.81; 95% CI, 1.38-2.37), being a female smoker (OR, 2.05; 95% CI, 1.51-2.80), and having a HAM-A score > 16 (OR, 2.18; 95% CI, 1.66-2.86). CONCLUSION: Smoking was found to be associated with anxiety symptoms, depressive symptoms, and poor school performance in Brazilian adolescents; and female smokers reported more suicidal ideation than male smokers.

Receiver operating characteristic curve analysis of the Child Behavior Checklist and Teacher's Report Form for assessing autism spectrum disorder in preschool-aged children.

BACKGROUND: Autism spectrum disorder (ASD) is a neurodevelopmental disorder characterized by social, behavioral, and communication impairments with an estimated prevalence of 1 in 68 school-aged children. There is a need for objective and easily applicable instruments for early identification of autistic children to enable initiation of early interventions during a very sensitive period of brain development and, consequently, optimize prognosis. Here, we tested the utility of the Child Behavior Checklist (CBCL) and the Caregiver-Teacher's Report Form (C-TRF) scales for assessing ASD in Brazil, where ASD screening research is emergent. SUBJECTS AND METHODS: A total of 70 children (2-5 years old, both sexes) were enrolled, including an ASD group (n=39) and a non-ASD control group (n=31). The preschool versions of the CBCL and C-TRF were applied. The CBCL and C-TRF results were compared between the ASD and non-ASD control groups with Mann-Whitney U tests and receiver operating characteristic analyses. RESULTS: The CBCL and C-TRF were found to have moderate accuracy for the dimensions withdrawn and autism spectrum problems, and to correlate with each other. CONCLUSION: The CBCL and C-TRF may aid in early ASD detection.

Attention-memory training yields behavioral and academic improvements in children diagnosed with attention-deficit hyperactivity disorder comorbid with a learning disorder.

BACKGROUND: Recent studies have suggested that children with attention-deficit hyperactivity disorder (ADHD) may benefit from computerized cognitive training. Therapy implementation is especially complicated when ADHD is associated with learning disorders (LDs). This study tested the efficacy of a computer-based cognitive training program, namely, computerized cognitive training (CCT), in children with ADHD comorbid with an LD (ADHD-LD), with or without psychostimulant medication. MATERIALS AND METHODS: After diagnostic evaluations, 27 children with ADHD-LD (8 unmedicated and 19 medicated) participated in CCT, which is intended to improve attention, memory, reasoning, visual processing, and executive functioning. The participants completed 24 1-hour sessions over 3 months. Neuropsychometric and standardized academic test results before and after training were compared to assess treatment efficacy. Shapiro-Wilk normality tests were applied, and subsequent Wilcoxon tests were used to identify significant differences in pre-versus post-training performance. RESULTS: After CAT, children diagnosed with ADHD-LD showed 1) improvements in trained skills, measured directly within the software and indirectly by external psychometric tests; 2) improvements in attention, memory, and some executive functioning; 3) improvements in academic performance, particularly in mathematics; and 4) reductions in maladaptive behavioral features. CONCLUSION: The present findings suggest that cognitive training programs should be explored further as potential adjunctive therapies to improve outcomes in children with ADHD-LD.