Summary Introduction The objective of this study is to assess the oral and maxillofacial characteristics of microcephalic children associated with congenital Zika syndrome (CZS). Methods A cross-sectional, observational study was carried out with 61 patients with microcephaly/CZS born between June 2015 and September 2017 (29 boys and 32 girls, average age of 22.8 months) and a control group with 58 non-CZS children born in the same period (25 boys and 33 girls, average age of 23.8 months). The functional clinical analysis considered the labial and lingual frena, tongue anterior projection, oral escape, palate form, and first tooth eruption. For the craniofacial analysis, facial anthropometric points and the cephalic perimeter at the time were measured. Demographic data were collected from medical records, and a clinical exam was performed in order to register the intrabuccal characteristics and craniofacial measures. The chi-square test and Student’s t-test were used with a significance level of 0.05. Results The narrow palate form, tongue anterior projection, oral escape, and late first tooth eruption were significantly more present in the group with microcephaly/CZS. As for the craniofacial analysis, face width (Bi-Zi), mandible width (Go-Go), height of face upper third (Tr-G), and monthly growth of cephalic perimeter were significantly smaller, whereas height of face lower third (Sn-Gn) was significantly bigger in the group with microcephaly/CZS (P < 0.05). Conclusion Children with microcephaly resulting from a congenital Zika infection showed functional, oral, and maxillofacial changes and smaller facial development in comparison with non-CZS children in the same age group.

transmission in Brazil was linked to a large outbreak of microcephaly but less is known about longer term anthropometric and neurological outcomes. We studied a cohort of infants born between October 31, 2015, and January 9, 2016, in a state maternity hospital, followed up for 101 ± 28 days by home visits. Microcephaly (< 2 standard deviations, Intergrowth standard) occurred in 62 of 412 (15%) births. Congenital Zika syndrome (CZS) was diagnosed in 29 patients. Among CZS patients, we observed a significant gain in anthropometric measures ( < 0.001) but no significant gain in percentile for these measures. The main neurological outcome was epilepsy, occurring in 48% of infants at a rate of 15.6 cases per 100 patient-months, frequently requiring multiple anti-seizure medications. The cumulative fatality rate was 7.4% (95% confidence interval: 2.1-23.4%). Health-care professionals should be alerted on the high risk of epilepsy and death associated with CZS in early infancy and the need to actively screen for seizures and initiate timely treatment.

Zika virus (ZIKV) infection during pregnancy causes congenital abnormalities, including microcephaly. However, rates vary widely, and the contributing risk factors remain unclear. We examined the serum antibody response to ZIKV and other flaviviruses in Brazilian women giving birth during the 2015–2016 outbreak. Infected pregnancies with intermediate or higher ZIKV antibody enhancement titers were at increased risk to give birth to microcephalic infants compared with those with lower titers (P &lt; 0.0001). Similarly, analysis of ZIKV-infected pregnant macaques revealed that fetal brain damage was more frequent in mothers with higher enhancement titers. Thus, features of the maternal antibodies are associated with and may contribute to the genesis of ZIKV-associated microcephaly.

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The authors report two cases of Brazilian children with most of the common syndromic features of Proteus syndrome, such as asymmetric overgrowth of tissues, skin abnormalities, hypotonia and mental retardation. In both patients, a refractory epilepsy, compatible with Ohtahara syndrome, as well as hemimegalencephaly, with asymmetric distribution of facial fat, were also diagnosed.

Alpha coma, an EEG pattern characterized by diffuse or widespread rhythmic activity in the alpha frequency band, is typically recorded in patients with profound coma and is frequently associated with severe neurological conditions. The most common etiologic factors of this pattern are hypoxic-ischemic encephalopathy, encephalitis, head trauma, metabolic disorders, and drug overdose. Reports of alpha coma pattern in children are relatively common. Clinical significance, both in children and adults, is variable, and highly dependent on etiology. The objective of this article is to report a clinical case of alpha coma pattern in a child with neuroblastoma. The EEG pattern was recorded during the evolution of treatment, secondary to complicating septic encephalopathy. The alpha coma pattern was replaced by a normal trace following a favorable outcome after sepsis resolution.

Background: This study was carried out in response to the Zika virus epidemic, which constituted a public health emergency, and to the 2019 WHO calling for strengthened surveillance for the early detection of related microcephaly. The main aim of the study was to phenotype the craniofacial morphology of microcephaly using a novel approach and new measurements, and relate the characteristics to brain abnormalities in Zika-infected infants in Brazil to improve clinical surveillance.Methods: 3D images of the face and the cranial vault of 44 Zika-infected infants and matched healthy controls were captured using a 3D stereophotogrammetry system. The CT scans of the brain of the infected infants were analysed. Principal component analysis (PCA) was applied to characterise the craniofacial morphology. In addition to the head circumference (HC), a new measurement, head height (HH), was introduced to measure the cranial vault. The level of brain abnormality present in the CT scans was assessed; the severity of parenchymal volume loss and ventriculomegaly was quantified. Student's t-test and Spearman's Rho statistical test have been applied.Findings: The PCA identified a significant difference (p &lt; 0.001) between the cranial vaults and the face of the Zika infants and that of the controls. Spearman's rank-order correlation coefficients show that the head height (HH) has a strong correlation (0.87 in Zika infants; 0.82 in controls) with the morphology of the cranial vaults, which are higher than the correlation with the routinely used head circumference (HC). Also, the head height (HH) has a moderate negative correlation (−0.48) with the brain abnormalities of parenchymal volume loss.Interpretation: It is discovered that the head height (HH) is the most sensitive and discriminatory measure of the severity of cranial deformity, which should be used for clinical surveillance of the Zika syndrome, evaluation of other craniofacial syndromes and assessment of various treatment modalities.

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