Objective To report radiological findings observed in computed tomography (CT) and magnetic resonance imaging (MRI) scans of the first cases of congenital infection and microcephaly presumably associated with the Zika virus in the current Brazilian epidemic.Design Retrospective study with a case series.Setting Association for Assistance of Disabled Children (AACD), Pernambuco state, Brazil.Participants 23 children with a diagnosis of congenital infection presumably associated with the Zika virus during the Brazilian microcephaly epidemic.Main outcome measures Types of abnormalities and the radiological pattern of lesions identified on CT and MRI brain scans.Results Six of the 23 children tested positive for IgM antibodies to Zika virus in cerebrospinal fluid. The other 17 children met the protocol criteria for congenital infection presumably associated with the Zika virus, even without being tested for IgM antibodies to the virus—the test was not yet available on a routine basis. Of the 23 children, 15 underwent CT, seven underwent both CT and MRI, and one underwent MRI. Of the 22 children who underwent CT, all had calcifications in the junction between cortical and subcortical white matter, 21 (95%) had malformations of cortical development, 20 (91%) had a decreased brain volume, 19 (86%) had ventriculomegaly, and 11 (50%) had hypoplasia of the cerebellum or brainstem. Of the eight children who underwent MRI, all had calcifications in the junction between cortical and subcortical white matter, malformations of cortical development occurring predominantly in the frontal lobes, and ventriculomegaly. Seven of the eight (88%) children had enlarged cisterna magna, seven (88%) delayed myelination, and six each (75%) a moderate to severe decrease in brain volume, simplified gyral pattern, and abnormalities of the corpus callosum (38% hypogenesis and 38% hypoplasia). Malformations were symmetrical in 75% of the cases.Conclusion Severe cerebral damage was found on imaging in most of the children in this case series with congenital infection presumably associated with the Zika virus. The features most commonly found were brain calcifications in the junction between cortical and subcortical white matter associated with malformations of cortical development, often with a simplified gyral pattern and predominance of pachygyria or polymicrogyria in the frontal lobes. Additional findings were enlarged cisterna magna, abnormalities of corpus callosum (hypoplasia or hypogenesis), ventriculomegaly, delayed myelination, and hypoplasia of the cerebellum and the brainstem.

Objective To describe the clinical, radiological, and electromyographic features in a series of children with joint contractures (arthrogryposis) associated with congenital infection presumably caused by Zika virus.Design Retrospective case series study.Setting Association for Assistance of Disabled Children, Pernambuco state, Brazil.Participants Seven children with arthrogryposis and a diagnosis of congenital infection presumably caused by Zika virus during the Brazilian microcephaly epidemic.Main outcome measures Main clinical, radiological, and electromyographic findings, and likely correlation between clinical and primary neurological abnormalities.Results The brain images of all seven children were characteristic of congenital infection and arthrogryposis. Two children tested positive for IgM to Zika virus in the cerebrospinal fluid. Arthrogryposis was present in the arms and legs of six children (86%) and the legs of one child (14%). Hip radiographs showed bilateral dislocation in seven children, subluxation of the knee associated with genu valgus in three children (43%), which was bilateral in two (29%). All the children underwent high definition ultrasonography of the joints, and there was no evidence of abnormalities. Moderate signs of remodeling of the motor units and a reduced recruitment pattern were found on needle electromyography (monopolar). Five of the children underwent brain computed tomography (CT) and magnetic resonance imaging (MRI) and the remaining two CT only. All presented malformations of cortical development, calcifications predominantly in the cortex and subcortical white matter (especially in the junction between the cortex and white matter), reduction in brain volume, ventriculomegaly, and hypoplasia of the brainstem and cerebellum. MRI of the spine in four children showed apparent thinning of the cord and reduced ventral roots.Conclusions Congenital Zika syndrome should be added to the differential diagnosis of congenital infections and arthrogryposis. The arthrogryposis was unrelated to the abnormalities of the joints themselves, but was possibly of neurogenic origin, with chronic involvement of central and peripheral motor neurones leading to deformities as a result of fixed postures in utero. Based on the neurophysiological observations, we suggest two possible mechanisms: tropism of neurones, with involvement of peripheral and central motor neurones, or a relation with vascular disorders.

BACKGROUND AND PURPOSE:Arthrogryposis is among the malformations of congenital Zika syndrome. Similar to the brain, there might exist a spectrum of spinal cord abnormalities. The purpose of this study was to explore and describe in detail the MR imaging features found in the spinal cords, nerve roots, and brains of children with congenital Zika syndrome with and without arthrogryposis.

Objective To compare initial brain computed tomography (CT) scans with follow-up CT scans at one year in children with congenital Zika syndrome, focusing on cerebral calcifications. Design Case series study. Setting Barão de Lucena Hospital, Pernambuco state, Brazil. Participants 37 children with probable or confirmed congenital Zika syndrome during the microcephaly outbreak in 2015 who underwent brain CT shortly after birth and at one year follow-up. Main outcome measure Differences in cerebral calcification patterns between initial and follow-up scans. Results 37 children were evaluated. All presented cerebral calcifications on the initial scan, predominantly at cortical-white matter junction. At follow-up the calcifications had diminished in number, size, or density, or a combination in 34 of the children (92%, 95% confidence interval 79% to 97%), were no longer visible in one child, and remained unchanged in two children. No child showed an increase in calcifications. The calcifications at the cortical-white matter junction which were no longer visible at follow-up occurred predominately in the parietal and occipital lobes. These imaging changes were not associated with any clear clinical improvements. Conclusion The detection of cerebral calcifications should not be considered a major criterion for late diagnosis of congenital Zika syndrome, nor should the absence of calcifications be used to exclude the diagnosis.

MDD seems to be associated with global HcV atrophy. Larger longitudinal follow-up studies designed to analyze the influence of sociodemographic variables on this relationship are required to yield better evidence about this topic.

Congenital Zika syndrome is an emergent cause of a congenital infectious disorder, resulting in severe damage to the central nervous system and microcephaly. Despite advances in understanding the pathophysiology of the disease, we still do not know all the mechanisms enrolled in the vertical transmission of the virus. As has already been reported in other types of congenital infectious disorders in dizygotic twin pregnancies, it is possible that the virus affects only one of the fetuses. In this article, we report on two cases of twin pregnancies exposed to the Zika virus, but with only one of the fetuses affected with microcephaly and brain damage. This indicates the urgent need for more studies regarding the pathophysiology of viral infection and the mechanisms involved in the natural protection against the virus.

The high prevalence of craniovertebral junction (CVJ) malformation among the population born in Northeastern Brazil has been described for a long time 1,2 . It is usually associated with brachycephalic biotype ( flat head), that is common in this region.Virchow' s seminal works described higher levels of basilar invagination among inhabitants of the Islands of the North Sea, the Netherlands and the Territory of Bremen (apud Silva) 1 . Silva 3 also described a doctoral thesis about this theme developed by Bogtstra in 1864 in the Netherlands. No other correlation was found between CVJ malformation and the Dutch.Despite the lack of any specific publication about this issue, it is widely accepted that the high prevalent rates of CVJ malformation among individuals born in Northeastern Brazil came from miscegenation with the Dutch during the colonial period of Brazil´s history 2 . The purpose of this article was to confront this paradigm against historical facts in order to verify the veracity of this assertion. DUTCH'S HABITS DURING THE COLONIAL PERIODDuring their brief stay in Northeastern Brazil, the Dutch preserved the same habits of their homeland. In contrast to the Portuguese, which adapted very well to the local Amerindian culture, the Dutch that came here were people from the city ABSTRACT The high prevalence of craniovertebral junction malformation in Northeastern Brazil is historically associated with brachycephalic biotype (flat head), also common in this region. It has been postulated that this trait was introduced to this region by the Dutch during the colonial period in Brazil's history. Based on the confrontation of this paradigm against some historical facts, the authors concluded that the brachycephalic phenotype was inherited from prehistoric ancestors (Amerindians) who were already living in this region when white European men arrived.Key words: platybasia, brachycephalic biotype, cranial junction malformation. RESUMOA alta prevalência de malformação da junção craniovertebral no Nordeste do Brasil é historicamente associada ao biótipo braquicefálico (cabeça chata), também comum nessa região. Postula-se que essa característica tenha sido introduzida na região pelos holandeses durante o período colonial da história do Brasil. Com base na confrontação desse paradigma com alguns fatos históricos, os autores concluem que o fenótipo braquicefálico foi herdado de ancestrais pré-históricos (ameríndios) que já habitavam a região no momento da chegada do homem branco europeu.Palavras-Chave: platibasia, biótipo braquicefálico, malformação da junção craniovertebral.

Chikungunya virus is capable of affecting the nervous system of children and adults. We describe a case of sepsis and encephalitis triggered by this agent in a newborn whose mother developed symptoms of acute infection 2 days before delivery. Consequently, the infant had severe encephalitis that evolved with postnatal-onset microcephaly, bilateral optic atrophy, epilepsy and cerebral palsy.