In the summer of 2015, several months after the Zika virus (ZIKV) epidemic was recognized
in northeastern Brazil, pediatric neurologists and obstetricians noticed an increased
number of fetuses with malformations and the Brazilian Ministry of Health soon reported
that the birth prevalence of microcephaly had increased from 0.6 to 2.8 per 10,000
live births in regions reporting ZIKV transmission. At the Hospital Infantil Albert
Sabin, Fortaleza-Brazil, we have accompanied 107 patients with Congenital Zika Syndrome,
91 with congenital microcephaly and 16 who were born with normal head circumference
and evolved with postnatal microcephaly. Our data demonstrate features of both fetal
brain disruption and primary cortical malformation, and define the nature and spectrum
of the disorder and five important points that together comprise a recognizable pattern
of disruption and malformation when seen together strongly support a diagnosis of
congenital Zika syndrome.
- (1)prenatal exposure to ZIKV carries a high risk of causing fetal anomalies including fetal brain disruption sequence, especially during the first and second trimesters, and that the risk may be higher among mothers with symptomatic ZIKV infections;
- (2)severe microcephaly with partially collapsed skull; thin cerebral cortices with subcortical calcifications; enlarged ventricles at birth but in 15 cases there was evolution to increase dilation and hypertensive hydrocephalus.
- (3)macular scarring and focal pigmentary retinal mottling;
- (4)congenital contractures and the occurrence of arthrogryposis
- (5)marked early hypertonia and symptoms of extrapyramidal involvement,with global neuropsychomotor development delays and incidence of epilepsy in about 30%.
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