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This article describes the Salvador Primary Care Longitudinal Study of Child Development (CohortDICa). The exposed group was defined by confirmation of Congenital Zika Syndrome (CZS) diagnosed through computed tomography, magnetic resonance or transfontanellar ultrasound. A random selection of the 169 exposed children led to a subgroup of 120 children who were paired with children from the Live Birth Information System, according to birthdate, residence in the same street or neighborhood, and gestational age, resulting in 115 subjects in the non-exposed group. Following recruitment and before the participants completed 42 months, three measures were applied to assess cognitive, motor, and language performance, corresponding to three home visits. Social characteristics of the families and children, and the neurocognitive development of the children will be compared across the CZS exposed group (n = 147), the typical children with no exposure to CZS (n = 115) and the STORCH exposed group (Syphilis, Toxoplasma gondii, Rubella, Cytomegalovirus, and Herpes simplex) (n = 20). Primary Health Care (PHC) should include long-term care strategies for the care of children and family members, and might benefit from the research, teaching, and extension activities provided in this study. In the face of the consequences of the Zika virus epidemic, an opportunity arose to intervene in the integrated care of child development within PHC, including, on an equal basis, typical children and those with delays or disabilities in the first six years of life.

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Maple urine syrup disease (MSUD) is an autosomal recessive disorder characterized by deficient activity of the branched-chain alpha ketoacid dehydrogenase (BCKAD) enzymatic complex due to biallelic variants in the alpha (BCKDHA) or beta (BCKDHB) subunits or the acyltransferase component (DBT). Treatment consists in leucine (LEU), isoleucine (ILE), and valine (VAL) (branched-chain amino acids) dietary restriction and strict metabolic control. to determine the characteristics of the Chilean cohort with MSUD currently in follow-up at Instituto de Nutrición y Tecnología de los Alimentos, during the 1990-2017 period Retrospective analytical study in 45 MSUD cases. Measured: biochemical parameters (LEU, ILE, and VAL), anthropometric evaluation, and neurocognitive development. In 18 cases undergoing genetic study were analyzed according to the gene and protein location, number of affected alleles, and type of posttranslational modification affected. Then, 45 patients with MSUD diagnosis were identified during the period: 37 were alive at the time of the study. Average diagnosis age was 71 ± 231 days. Average serum diagnosis LEU concentrations: 1.463 ± 854.1 µmol/L, VAL 550 ± 598 µmol/L and ILE 454 ± 458 µmol/L. BCKDHB variants explain 89% cases, while BCKDHA and DBT variants explain 5.5% of cases each. Variants p.Thr338Ile in BCKDHA, p.Pro240Thr and p.Ser342Asn in BCKDHB have not been previously reported in literature. Average serum follow-up LEU concentrations were 252.7 ± 16.9 µmol/L in the <5 years group and 299 ± 123.2 µmol/L in ≥5 years. Most cases presented some degree of developmental delay. Early diagnosis and treatment is essential to improve the long-term prognosis. Frequent blood LEU measurements are required to optimize metabolic control and to establish relationships between different aspects analyzed.

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BACKGROUND: Agenesis of the corpus callosum can occur isolated or as part of a complex congenital syndrome. Patients with isolated agenesis of the corpus callosum may present with severe intellectual disability, although a proportion of affected individuals develop normal intelligence. However, even in patients with no apparent deficits, subtle neuropsychological alterations may occur as the cognitive demand increases with age. Hence, patients with this deffect require a strict follow-up during their postnatal life. Thus, physicians require a better knowledge of the cognitive features of agenesis of the corpus callosum to improve their approach to this cerebral malformation. Here, we report an illustrative case of a school-age child with isolated agenesis of the corpus callosum and normal intelligence. We also provide a literature review about the postnatal screening of neurocognitive deficits in patients with agenesis of the corpus callosum. CASE PRESENTATION: An 8-year-old Hispanic boy with total agenesis of the corpus callosum attended for medical follow-up. The defect was identified during the neonatal period by cranial ultrasonography and brain computed tomography scan. However, he did not present any craniofacial or non-cerebral malformation suggestive of a congenital syndrome. Furthermore, he showed no neuropsychiatric disorder or intellectual disability during his early childhood. At the age of 4, he was subjected to a control brain magnetic resonance imaging that showed total agenesis of the corpus callosum and colpocephaly. At his arrival, a neurological examination was normal with no signs of intracranial hypertension. His intelligence quotient was unaltered and he scored normal in the Mini-Mental State Examination test. The literature reviewed here suggested that patients with agenesis of the corpus callosum require a strict neurocognitive follow-up during postnatal life, as they may present neuropsychological deficits during adolescence, when development of the corpus callosum is completed and there is maximum reliance on this structure. Thus, our patient was scheduled for future annual neurocognitive testing. CONCLUSIONS: Isolated agenesis of the corpus callosum is not innocuous, and patients with this defect require a strict neurocognitive follow-up. We provide an informative reference tool useful for the postnatal neuropsychological screening of patients with isolated agenesis of the corpus callosum.

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Abstract Neurological Soft Signs (NSS) are indicative of generalized disruptions in neurological networks of cortico-subcortical areas. Their presence leads to problems in children's cognitive development with future academic repercussions. The presence of NSS was assessed and compared in 144 children aged 6 to 11 years of low-medium socioeconomic status from Mexico City and the metropolitan area through the Infant Neuropsychological Maturity Questionnaire (CUMANIN, for its Spanish acronym) and the School Neuropsychological Maturity Questionnaire (CUMANES, for its Spanish acronym). Results indicate significant differences by sex. Girls showed better performance in word articulation. In groups by age, significant differences were found in leximetric-comprehension, visual perception and executive function-errors. Participants showed the presence of developmental NSS which include: language (articulation disorders, oral and written language disturbances, difficulty finding words), psychomotricity, visual perception and other cognitive functions. There are several factors related to those impairments such as age, socioeconomic context and critical stages in child's development. Identification and early diagnosis can reduce the risk of school failure.

Resumo Os Sinais Neurológico Sutis (SNS) são indicadores de interrupções generalizadas nas redes de trabalho neural de áreas córtico-subcorticais, cuja presença leva a problemas no desenvolvimento neurocognitivo da criança que representam repercussões acadêmicas negativas. Na presente pesquisa, foi avaliada e comparada a presença de SNS com os processos cognitivos de 144 participantes mexicanos de estrato socioeconómico médio-baixo com idades entre 6 e 11 anos, sem antecedentes neurológicos ou psiquiátricos, por meio dos Cuestionarios de Madurez Neuropsicológica (CUMANIN) e Madurez Neuropsicológica Escolar (CUMANES). Os resultados indicaram diferenças significativas por gênero, já que as meninas apresentaram melhor desempenho na articulação de palavras. Nos grupos por idade, as diferenças significativas foram encontradas na velocidade de leitura e compreensão, visuopercepção e função executiva e erros. Em geral, os participantes mostraram presença de SNS de desenvolvimento, que incluem interferências na linguagem (problemas articulatórios, alterações da linguagem oral e escrita, dificuldade para encontrar palavras), psicomotricidade, visuopercepção e outras funções cognitivas. Ao final, conclui-se que a identificação e o diagnóstico precoce dos SNS permite diminuir o risco de fracasso escolar.

Resumen Los Signos Neurológicos Blandos (SNB) son indicativos de interrupciones generalizadas en las redes de trabajo neuronal de áreas cortico-subcorticales, cuya presencia conlleva a problemas en el desarrollo neurocognitivo del niño que representan repercusiones académicas negativas. En la presente investigación se evaluó y comparó la presencia de SNB con los procesos cognitivos de 144 participantes mexicanos de estrato socioeconómico medio-bajo con edades entre los 6 y 11 años sin antecedentes neurológicos o psiquiátricos por medio de los Cuestionarios de Madurez Neuropsicológica (CUMANIN) y Madurez Neuropsicológica Escolar (CUMANES). Los resultados indicaron diferencias significativas por sexo, ya que las niñas presentaron mejor desempeño en la articulación de palabras. En los grupos por edad, las diferencias significativas se encontraron en leximetría-comprensión, visopercepción y función ejecutiva-errores; y, en general, los participantes mostraron presencia de SNB de desarrollo, que incluyen afectaciones en: lenguaje (problemas articulatorios, alteraciones del lenguaje oral y escrito, dificultad para encontrar palabras), psicomotricidad, visopercepción y otras funciones cognitivas. Al final, se concluye que la identificación y diagnóstico temprano de los SNB permite disminuir el riesgo de fracaso escolar.

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PURPOSE: The Early Life Exposure in Mexico to ENvironmental Toxicants (ELEMENT) Project is a mother-child pregnancy and birth cohort originally initiated in the mid-1990s to explore: (1) whether enhanced mobilisation of lead from maternal bone stores during pregnancy poses a risk to fetal and subsequent offspring neurodevelopment; and (2) whether maternal calcium supplementation during pregnancy and lactation can suppress bone lead mobilisation and mitigate the adverse effects of lead exposure on offspring health and development. Through utilisation of carefully archived biospecimens to measure other prenatal exposures, banking of DNA and rigorous measurement of a diverse array of outcomes, ELEMENT has since evolved into a major resource for research on early life exposures and developmental outcomes. PARTICIPANTS: n=1643 mother-child pairs sequentially recruited (between 1994 and 2003) during pregnancy or at delivery from maternity hospitals in Mexico City, Mexico. FINDINGS TO DATE: Maternal bone (eg, patella, tibia) is an endogenous source for fetal lead exposure due to mobilisation of stored lead into circulation during pregnancy and lactation, leading to increased risk of miscarriage, low birth weight and smaller head circumference, and transfer of lead into breastmilk. Daily supplementation with 1200 mg of elemental calcium during pregnancy and lactation reduces lead resorption from maternal bone and thereby, levels of circulating lead. Beyond perinatal outcomes, early life exposure to lead is associated with neurocognitive deficits, behavioural disorders, higher blood pressure and lower weight in offspring during childhood. Some of these relationships were modified by dietary factors; genetic polymorphisms specific for iron, folate and lipid metabolism; and timing of exposure. Research has also expanded to include findings published on other toxicants such as those associated with personal care products and plastics (eg, phthalates, bisphenol A), other metals (eg, mercury, manganese, cadmium), pesticides (organophosphates) and fluoride; other biomarkers (eg, toxicant levels in plasma, hair and teeth); other outcomes (eg, sexual maturation, metabolic syndrome, dental caries); and identification of novel mechanisms via epigenetic and metabolomics profiling. FUTURE PLANS: As the ELEMENT mothers and children age, we plan to (1) continue studying the long-term consequences of toxicant exposure during the perinatal period on adolescent and young adult outcomes as well as outcomes related to the original ELEMENT mothers, such as their metabolic and bone health during perimenopause; and (2) follow the third generation of participants (children of the children) to study intergenerational effects of in utero exposures. TRIAL REGISTRATION NUMBER: NCT00558623.

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BACKGROUND: Adolescent motherhood remains common in developing countries and is associated with risk factors that adversely impact infant neurodevelopment, including poverty, low maternal education, and increased maternal psychopathology. Yet, no published work has assessed how these factors affect early brain development in developing countries. METHODS: This pilot study examined effects of maternal psychopathology and education on early neurocognitive development in a sample of adolescent mothers (N = 50, final n = 31) and their infants living in poverty in São Paulo, Brazil. Maternal symptoms of anxiety, depression, and attention-deficit/hyperactivity disorder and education level were assessed during pregnancy. Infant neurocognitive development was assessed at 6 months of age, with oscillatory power and functional connectivity in the theta (4-6 Hz), alpha (6-9 Hz), and gamma (30-50 Hz) frequencies derived from resting-state electroencephalography; temperament (negative affect, attention, and regulation); and cognitive, language, and motor skills. Cluster-based permutation testing and graph-theoretical methods were used to identify alterations in oscillatory power and connectivity that were associated with maternal psychopathology and education. Correlations between power and connectivity alterations were examined in relation to infants' overt cognitive behavioral abilities. RESULTS: Increased maternal anxiety and lower maternal education were associated with weaker oscillatory connectivity in alpha-range networks. Infants with the weakest connectivity in the alpha network associated with maternal anxiety also showed the lowest cognitive ability. Greater maternal anxiety and attention-deficit/hyperactivity disorder were associated with increased absolute and relative theta power. CONCLUSIONS: Our findings highlight the importance of addressing maternal psychopathology and improving education in poor adolescent mothers to prevent negative effects on infant neurodevelopment.

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OBJECTIVE: To determine demographic, maternal, and child factors associated with socioemotional (SE) problems and chronic stress in 1-year-old children. STUDY DESIGN: This was a prospective, longitudinal, community-based study, which followed mother-infant dyads (n = 1070; representative of race, education, and income status of Memphis/Shelby County, Tennessee) from midgestation into early childhood. Child SE development was measured using the Brief Infant-Toddler Social and Emotional Assessment in all 1097 1-year-olds. Chronic stress was assessed by hair cortisol in a subsample of 1-year-olds (n = 297). Multivariate regression models were developed to predict SE problems and hair cortisol levels. RESULTS: More black mothers than white mothers reported SE problems in their 1-year-olds (32.9% vs 10.2%; P < .001). In multivariate regression, SE problems in blacks were predicted by lower maternal education, greater parenting stress and maternal psychological distress, and higher cyclothymic personality score. In whites, predictors of SE problems were Medicaid insurance, higher maternal depression score at 1 year, greater parenting stress and maternal psychological distress, higher dysthymic personality score, and male sex. SE problem scores were associated with higher hair cortisol levels (P = .01). Blacks had higher hair cortisol levels than whites (P < .001). In the entire subsample, increased hair cortisol levels were associated with higher parenting stress (P = .001), lower maternal depression score (P = .01), lower birth length (P < .001), and greater length at 1 year of age (P = .003). CONCLUSION: Differences in maternal education, insurance, mental health, and early stress may disrupt SE development in children. Complex relationships between hair cortisol level in 1-year-olds and maternal parenting stress and depression symptoms suggest dysregulation of the child's hypothalamic-pituitary-adrenal axis.

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There have been indications that intrauterine and early extrauterine growth can influence childhood mental and motor function. The objective of the present study was to evaluate the influence of intrauterine growth restriction and early extrauterine head growth on the neurodevelopmental outcome of monozygotic twins. Thirty-six monozygous twin pairs were evaluated at the corrected age of 12 to 42 months. Intrauterine growth restriction was quantified using the fetal growth ratio. The effects of birth weight ratio, head circumference at birth and current head circumference on mental and motor outcomes were estimated using mixed-effect linear regression models. Separate estimates of the between (interpair) and within (intrapair) effects of each measure on development were thus obtained. Neurodevelopment was assessed with the Bayley Scales of Infant Development, 2nd edition, by a psychologist blind to the exposure. A standardized neurological examination was performed by a neuropediatrician who was unaware of the exposures under investigation. After adjustment, birth weight ratio and head circumference at birth were not associated with motor or mental outcomes. Current head circumference was associated with mental but not with motor outcomes. Only the intrapair twin effect was significant. An increase of 1 cm in current head circumference of one twin compared with the other was associated with 3.2 points higher in Mental Developmental Index (95 percentCI = 1.06-5.32; P < 0.03). Thus, no effect of intrauterine growth was found on cognition and only postnatal head growth was associated with cognition. This effect was not shared by the co-twin.

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