Abstract

AbstractWe report a male infant, twin A of a dichorionic pregnancy, with a fetal ultrasound and subsequent MRI at 29 weeks of gestation revealing a small cranium (26 cm, < 3rd percentile for gestation) and marked ex vacuo ventriculomegaly. Twin B had normal ultrasound and brain MRI. Newborn exam was unremarkable and his weight and head circumference were both at 5th percentile. Placental pathology findings included focal acute vasculitis and chronic villitis. Postnatal brain MRI showed significant cerebral volume loss with extensive large cystic parietal encephalomalacia and diffuse cerebral cortical dysplasia (polymicrogyria). Common congenital infectious diseases investigation on maternal serum was normal. Twin A was admitted for bronchiolitis and possible seizure at 3 months of age. At this time, he had microcephaly and was not tracking light. Ophthalmology exam showed markedly abnormal macula with diffuse pigment changes and scarring that extended between the full arcades bilaterally. Congenital lymphocytic choriomeningitis virus (LCMV) infection was confirmed with negative IgM titer and highly positive IgG titer of greater than 1:256. Serologic tests for LCMV should be considered in infants who have central nervous system and retinal involvement but negative TORCH studies.

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