Risk of autism spectrum disorder in children with infantile epileptic spasms syndrome: a retrospective study in a single center in Brazil.

OBJECTIVE: This study aimed to investigate the prevalence of autism spectrum disorder and its possible correlations with clinical characteristics in patients with infantile epileptic spasms syndrome in a single center in Brazil. METHODS: This retrospective cross-sectional study examined 53 children with the diagnosis of infantile epileptic spasms syndrome prior to an autism spectrum disorder assessment. Participants were divided into two groups based on the presence or absence of autism spectrum disorder. Available variables (sex, medications, median age at onset of infantile epileptic spasms syndrome, and presence of comorbidities) were compared using Mann-Whitney U or chi-square tests. RESULTS: Among the included patients, 12 (23 %) were diagnosed with autism spectrum disorder, corresponding to a relative risk of 0.29 (95 % confidence interval 0.174-0.492). The age at the first seizure ranged from 3 to 15 months, with a mean of 6.65 months. This age significantly differed between participants with autism spectrum disorder (10.58 months) and those without (5.43 months), p<0.001. CONCLUSION: Children with infantile epileptic spasms syndrome have a higher risk of being diagnosed with autism spectrum disorder. Later age of onset and period of spasm occurrence might be predisposing risk factors.

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.

Psychobehavioral factors and family functioning in mucopolysaccharidosis: preliminary studies.

Introduction: Mucopolysaccharidoses (MPS) constitute a group of progressive and multisystemic inherited metabolic diseases that profoundly affect both the mental health of patients and the wellbeing of their families. This study aims to evaluate the impact of MPS on family functioning and related factors. Methods and results: Twenty-five patients with MPS, including types I (n = 4), II (n = 11), IIIB (n = 2), IVA (n = 3), and VI (n = 5), and their families participated in this study. The mean patient age was 13 years [standard deviation (SD): 7.7 years]. Behavioral and emotional problems were noted in 9.1% of all patients. While the type of MPS did not directly influence mental problems, the presence of neuronal involvement did (p = 0.006). Patients with MPS III exhibited difficulties primarily in emotional areas, conduct, hyperactivity, and peer problems. Importantly, both patients with MPS II and those with MPS III experienced a significant impact on communication [mean scores for communication domain: MPS II, 35.6 (SD: 24.3); MPS III, 35.0 (SD: 22.6)]; poorer communication was directly linked to worse adaptive behavior (p = 0.012), and worse adaptive behavior was associated with lower quality of life (p = 0.001). Quality of life and caregiver burden among family members did not significantly differ across MPS types; however, higher caregiver burden was negatively associated with quality of life (p = 0.002). Concerning family functioning, the most impacted domains included independence, intellectual/cultural orientation, activity/recreation, and expressiveness. Domain scores did not vary based on MPS type, treatment, or neurological involvement. Quality-of-life scores were positively associated with the cultural/intellectual domain score. Conclusion: The impacts of quality of life and family extend beyond clinical characteristics and MPS type, strongly influenced by patient cognition and communication, as well as type of family functioning, especially those with greater cultural/intellectual skills of their family members. A multidisciplinary approach addressing the broader needs of individuals with MPS becomes essential. Techniques aimed at improving communication, including prompt interventions such as speech therapy and augmentative and alternative communication strategies, can contribute to overall family functioning improvement.

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.

Neurological, neurobehavioral, and radiological alterations in patients with mucopolysaccharidosis III (Sanfilippo's syndrome) in Brazil.

Mucopolysaccharidosis type III (MPS III) or Sanfilippo syndrome is the most common form of MPS, in which neurological involvement in all stages of the disease is prominent. The current study aimed to comprehensively describe the neurological profile of children and adolescents with MPS III who visited the largest pediatric hospital in South America. A prospective/retrospective cohort analysis was performed on 10 patients with MPS III from eight unrelated families. Most patients <12 months of age had achieved development milestones within the expected range for their age, with delay in walking independently and first single word acquisition. Behavioral symptoms were reported in seven patients. Eight patients (80%) developed profound intellectual disabilities. Six patients (60%) had epilepsy, among whom 75% had their first seizure between 2 and 4 years of age; the frequency of which increased with age. Monotherapy was effective in 60% of patients. Two patients, both aged <8 years, had normal baseline electroencephalographic activity. Epileptiform activity was observed in three patients. Cortical atrophy was visualized using magnetic resonance imaging in 71% patients; all but one of these patients were aged >6 years. Neurological abnormalities increased in prevalence and severity with age. Anti-seizure drug resistance was uncommon. Dysmorphological and systemic manifestations were uncommon and mild and did not correlate with neurological involvement. Despite high allelic heterogeneity, neurodegeneration was similar among all patients. Overall, these data contribute to the scarce literature from developing countries.

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.

Neurological Characteristics of Pediatric Glycogen Storage Disease.

Glycogen storage diseases (GSD) encompass a group of rare inherited diseases due dysfunction of glycogen metabolism. Hypoglycemia is the most common primary manifestation of GSD, and disturbances in glucose metabolism can cause neurological damage. The aims of this study were to first investigate the metabolic, genetic, and neurological profiles of children with GSD, and to test the hypothesis whether GSD type I would have greater neurological impact than GSD type IX. A cross-sectional study was conducted with 12 children diagnosed with GSD [Types: Ia (n=5); 1, Ib (n=1); 4, IXa (n=5); and 1, IXb (n=1)]. Genetic testing was conducted for the following genes using multigene panel analysis. The biochemical data and magnetic resonance imaging of the brain presented by the patients were evaluated. The criteria of adequate metabolic control were adopted based on the European Study on Glycogen Storage Disease type I consensus. Pathogenic mutations were identified using multigene panel analyses. The mutations and clinical chronology were related to the disease course and neuroimaging findings. Adequate metabolic control was achieved in 67% of patients (GSD I, 43%; GSD IX, 100%). Fourteen different mutations were detected, and only two co-occurring mutations were observed across families (G6PC c.247C>T and c.1039C>T). Six previously unreported variants were identified (5 PHKA2; 1 PHKB). The proportion of GSD IX was higher in our cohort compared to other studies. Brain imaging abnormalities were more frequent among patients with GSD I, early-symptom onset, longer hospitalization, and inadequate metabolic control. The frequency of mutations was similar to that observed among the North American and European populations. None of the mutations observed in PHKA2 have been described previously. Therefore, current study reports six GSD variants previously unknown, and neurological consequences of GSD I. The principal neurological impact of GSD appeared to be related to inadequate metabolic control, especially hypoglycemia.

GABAA receptor encephalitis associated with human parvovirus B19 virus infection: Case report.

RATIONALE: Human parvovirus B19 (B19) infection can produce a spectrum of clinical syndromes, including neurological manifestations, most notably encephalitis. Although symptoms suggestive of autoimmune disease in patients with B19 infection have been previously described, a clear association of autoimmune encephalitis with B19 infection has yet to be established. PATIENT CONCERNS: We describe the case of a 6-year-old boy who was hospitalized due to status epilepticus, which evolved to super-refractory status epilepticus that was only mildly responsive to anticonvulsant drugs. DIAGNOSIS: A cerebrospinal fluid study identified slight pleocytosis and B19 positivity. A subsequent autoimmunity cerebrospinal fluid study revealed the presence of anti-γ-aminobutyric acid type A (GABAA) receptor antibodies. INTERVENTIONS: After pulse therapy with methylprednisolone and continuous therapy with prednisolone with cyclosporine, the patient experiencing seizure persistence with disordered motor function manifestations and only minor improvement in consciousness, and so, plasmapheresis was performed. With continued immunosuppressive treatments with cyclosporine and prednisolone, the patient's clinical picture showed progressive improvement, with good control of seizures. Although the patient tolerated withdrawal of the anticonvulsant drugs well, he developed seizures when corticosteroid therapy withdrawal was attempted, so was started on azathioprine. OUTCOMES: After immunosuppressive therapy, the patient evolved with complete remission of symptoms, normal neurological examination and age-appropriate neuropsychomotor development. LESSONS: The present case characteristics, together with previous findings, support the hypothesis that autoimmunity may be triggered by extensive antigen release due to degeneration of infected neurons. This case highlights the importance of early clinical suspicion and treatment.

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.

ADHD Comorbid With Major Depression on Parents and Teachers Perceptions.

Objective: Examine the Strengths and Difficulties Questionnaire (SDQ) responses of parents and teachers for children with ADHD comorbid with major depressive disorder (MDD), with an emphasis on determining how well the respondent groups' responses correlate, and how well the results obtained perform as predictors of clinical diagnosis. Method: The SDQ was completed by parents and teachers of (n = 215 participants, 7-12 years old) in ADHD, MDD, ADHD + MDD, and healthy control groups. Agreement between parent and teacher SDQs and their concordance with Diagnostic and Statistical Manual of Mental Disorders (4th ed.; DSM-IV) diagnoses were assessed. Receiver operating characteristic (ROC) and Kappa concordance analyses were used to compare the groups with the health control group. Results: The comorbid group presented greater impairments than the ADHD, MDD, and control groups (p < .001). Conclusion: The presence of psychiatric comorbidity causes greater impairment for school children with ADHD. The SDQ has good sensitivity for detecting these children and correlates well with DSM diagnosis.

Pediatric Anti-N-methyl-D-aspartate Receptor Encephalitis Mimicking Glutaric Aciduria Type 1: A Case Report.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an immune-mediated disease that induces a wide spectrum of symptoms, especially in toddlers. These include acute-onset movement disorders, with neurological regression, and other associated neurological symptoms. Anti-NMDAR encephalitis remains a diagnostic challenge, especially in toddlers, with better prognosis associated with early treatment. We report the case of a 15-months-old boy who initially presented with vomiting and later with acute-onset dystonia after the administration of antiemetics. Within 14 days, the patient developed neuropsychomotor developmental regression and worsening dystonia. After ruling out an acute dystonic reaction and glutaric acidemia type 1 (GA-1), a final diagnosis of anti-NMDAR encephalitis was made. The patient responded well to immunomodulatory therapy. The present case underscores the importance of early treatment for patient prognosis and of including anti-NMDAR encephalitis in the differential diagnosis of acute-onset movement disorders.

Receiver Operating Characteristic Curve Analysis of Screening Tools for Bipolar Disorder Comorbid With ADHD in Schoolchildren.

Objective: We compared Child Behavior Checklist (CBCL)-AAA (Attention Problems, Aggressive Behavior, and Anxious/Depressed) and Parent-Young Mania Rating Scale (P-YMRS) profiles in Brazilian children with ADHD, pediatric-onset bipolar disorder (PBD), and PBD + ADHD. Method: Following analyses of variance or Kruskal-Wallis tests with multiple-comparison Least Significant Difference (LSD) or Dunn's Tests, thresholds were determined by Mann-Whitney U Tests and receiver operating characteristic (ROC) plots. Results: Relative to ADHD, PBD and PBD + ADHD groups scored higher on the Anxious/Depressed, Thought Problems, Rule-Breaking, and Aggressive Behavior subscales and Conduct/Delinquency Diagnostic Scale of the CBCL; all three had similar attention problems. The PBD and PBD + ADHD groups scored higher than the ADHD and healthy control (HC) groups on all CBCL problem scales. The AAA-profile ROC had good diagnostic prediction of PBD + ADHD. PBD and PBD-ADHD were associated with (similarly) elevated P-YMRS scores. Conclusion: The CBCL-PBD and P-YMRS can be used to screen for manic behavior and assist in differential diagnosis.

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.

Phenotypic and genotypic aspects of Townes-Brock syndrome: case report of patient in southern Brazil with a new SALL1 hotspot region nonsense mutation.

BACKGROUND: Townes-Brocks syndrome (TBS) is a rare autosomal dominant condition characterized by renal, anal, limb, and auditory abnormalities. TBS diagnosis can be challenging in settings where genetic analysis is not readily available. TBS traits overlap with those of Goldenhar and VACTERL syndromes. CASE PRESENTATION: Here, we present the case of a 5-year-old Brazilian boy born with an anorectal abnormality, limb and external ears malformations, genitourinary anomalies, and a congenital heart defect. Genetic analysis revealed a SALL1 nonsense mutation. The case is discussed in the context of the current literature. CONCLUSIONS: Because of the variability in TBS clinical presentation, genetic analysis is key to the differential diagnosis of TBS relative to phenotypically similar syndromes.

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.

Brazilian adaptation of the Functioning after Pediatric Cochlear Implantation (FAPCI): comparison between normal hearing and cochlear implanted children / Adaptação brasileira do questionário Functioning Inventory after Pediatric Cochlear Implantation (FAPCI): comparação entre crianças com audição normal e com implante coclear

OBJECTIVE: Enabling development of the ability to communicate effectively is the principal objective of cochlear implantation (CI) in children. However, objective and effective metrics of communication for cochlear-implanted Brazilian children are lacking . The Functioning after Pediatric Cochlear Implantation (FAPCI), a parent/caregiver reporting instrument developed in the United States, is the first communicative performance scale for evaluation of real-world verbal communicative performance of 2-5-year-old children with cochlear implants. The primary aim was to cross-culturally adapt and validate the Brazilian-Portuguese version of the FAPCI. The secondary aim was to conduct a trial of the adapted Brazilian-Portuguese FAPCI (FAPCI-BP) in normal hearing (NH) and CI children. METHODS: The American-English FAPCI was translated by a rigorous forward-backward process. The FAPCI-BP was then applied to the parents of children with NH (n = 131) and CI (n = 13), 2-9 years of age. Test-retest reliability was verified. RESULTS: The FAPCI-BP was confirmed to have excellent internal consistency (Cronbach's alpha > 0.90). The CI group had lower FAPCI scores (58.38 ± 22.6) than the NH group (100.38 ± 15.2; p < 0.001, Wilcoxon test). CONCLUSION: The present results indicate that the FAPCI-BP is a reliable instrument. It can be used to evaluate verbal communicative performance in children with and without CI. The FAPCI is currently the only psychometrically-validated instrument that allows such measures in cochlear-implanted children. .

OBJETIVO: O principal objetivo do implante coclear (IC) em crianças é permitir o desenvolvimento da capacidade de se comunicar efetivamente. Contudo, não há objetivo nem parâmetros efetivos de comunicação para crianças brasileiras com o implante coclear. O Functioning after Pediatric Cochlear Implantation (FAPCI), instrumento de relato dos pais/prestadores de cuidados desenvolvido nos Estados Unidos, é a primeira escala de desempenho para avaliação do desempenho comunicativo verbal no mundo real de crianças de 2-5 anos com implantes cocleares. Nosso principal objetivo era adaptar e validar a versão do FAPCI em português do Brasil de forma transcultural. Nosso objetivo secundário era fazer um teste da versão do FAPCI adaptada para o português do Brasil (FAPCI-PB) com grupos de crianças com audição normal (AN) e IC. MÉTODOS: O FAPCI em inglês americano foi traduzido por um processo rigoroso de tradução e retrotradução. O FAPCI-PB foi, então, aplicado aos pais das crianças com AN (n = 131) e IC (n = 13) de 2-9 anos. Foi verificada a confiabilidade da reaplicação do teste. RESULTADOS: Confirmou-se que o FAPCI-PB tem excelente coerência interna (alfa de Cronbach > 0,90). O grupo com IC apresentou menores pontuações no FAPCI (58,38 ± 22,6) do que o grupo com AN (100,38 ± 15,2; p < 0,001, teste de Wilcoxon). CONCLUSÃO: Esses resultados indicam que o FAPCI-PB é um instrumento confiável. Pode ser usado para avaliar o desempenho comunicativo verbal em crianças com e sem IC. O FAPCI é, atualmente, o único instrumento validado psicometricamente que possibilita essas medições em crianças com implante coclear. .

Brazilian adaptation of the Functioning after Pediatric Cochlear Implantation (FAPCI): comparison between normal hearing and cochlear implanted children.

OBJECTIVE: Enabling development of the ability to communicate effectively is the principal objective of cochlear implantation (CI) in children. However, objective and effective metrics of communication for cochlear-implanted Brazilian children are lacking. The Functioning after Pediatric Cochlear Implantation (FAPCI), a parent/caregiver reporting instrument developed in the United States, is the first communicative performance scale for evaluation of real-world verbal communicative performance of 2-5-year-old children with cochlear implants. The primary aim was to cross-culturally adapt and validate the Brazilian-Portuguese version of the FAPCI. The secondary aim was to conduct a trial of the adapted Brazilian-Portuguese FAPCI (FAPCI-BP) in normal hearing (NH) and CI children. METHODS: The American-English FAPCI was translated by a rigorous forward-backward process. The FAPCI-BP was then applied to the parents of children with NH (n=131) and CI (n=13), 2-9 years of age. Test-retest reliability was verified. RESULTS: The FAPCI-BP was confirmed to have excellent internal consistency (Cronbach's alpha > 0.90). The CI group had lower FAPCI scores (58.38 ± 22.6) than the NH group (100.38 ± 15.2; p<0.001, Wilcoxon test). CONCLUSION: The present results indicate that the FAPCI-BP is a reliable instrument. It can be used to evaluate verbal communicative performance in children with and without CI. The FAPCI is currently the only psychometrically-validated instrument that allows such measures in cochlear-implanted children.

Depressive symptoms and suicidal ideation in adolescents accompanying a parent in recyclable trash collection.

The relationship between mental health and poverty has been well documented in adults. However, few studies have addressed how low socioeconomic status and psychosocial vulnerabilities may influence depressive symptoms in adolescents. The current study was carried out in a non-randomly selected sample of 239 adolescents whose parents work as ragpickers (waste collectors for recycling) in Brazil. In-person interviews were conducted, and the presence of depressive symptoms and suicidal ideation were assessed using the Children's Depression Inventory (CDI). We observed that 23% (CI ± 5.34) of the adolescents presented with depressive symptoms and 35% (CI ± 6.05) had suicidal ideation. Fatigue or loss of energy (p = .012) and irritable mood (p = .013) were significantly higher among boys than girls according to DSM-IV criteria. However, we found no gender differences in DSM-IV criteria for Major Depressive Disorders (MDD) or Dysthymic Disorder (DD) in diminished interest or pleasure, weight loss or weight gain, decreased appetite, sleep problems, feelings of worthlessness, diminished concentration or ability to think, recurrent thoughts of death, suicidal ideation, or low self-esteem. There were no significant gender differences in total CDI score, however a greater percentage of girls presented with depressed mood than boys (29.9% vs. 17.1%, p < .05).

Effects of sleep deprivation on central auditory processing.

BACKGROUND: Sleep deprivation is extremely common in contemporary society, and is considered to be a frequent cause of behavioral disorders, mood, alertness, and cognitive performance. Although the impacts of sleep deprivation have been studied extensively in various experimental paradigms, very few studies have addressed the impact of sleep deprivation on central auditory processing (CAP). Therefore, we examined the impact of sleep deprivation on CAP, for which there is sparse information. In the present study, thirty healthy adult volunteers (17 females and 13 males, aged 30.75±7.14 years) were subjected to a pure tone audiometry test, a speech recognition threshold test, a speech recognition task, the Staggered Spondaic Word Test (SSWT), and the Random Gap Detection Test (RGDT). Baseline (BSL) performance was compared to performance after 24 hours of being sleep deprived (24hSD) using the Student's t test. RESULTS: Mean RGDT score was elevated in the 24hSD condition (8.0±2.9 ms) relative to the BSL condition for the whole cohort (6.4±2.8 ms; p=0.0005), for males (p=0.0066), and for females (p=0.0208). Sleep deprivation reduced SSWT scores for the whole cohort in both ears [(right: BSL, 98.4%±1.8% vs. SD, 94.2%±6.3%. p=0.0005)(left: BSL, 96.7%±3.1% vs. SD, 92.1%±6.1%, p<0.0001)]. These effects were evident within both gender subgroups [(right: males, p=0.0080; females, p=0.0143)(left: males, p=0.0076; females: p=0.0010). CONCLUSION: Sleep deprivation impairs RGDT and SSWT performance. These findings confirm that sleep deprivation has central effects that may impair performance in other areas of life.