Focal epilepsy features in a child with Congenital Zika Syndrome

Abstract

Zika virus (ZIKV) is a mosquito-borne, single-stranded DNA flavivirus that is teratogenic and neurotropic. Similar to the teratogenic effects of other TORCH infections, ZIKV infection during pregnancy can have an adverse impact on fetal and neonatal development. Epilepsy is detected in 48-96% of children with Congenital Zika Syndrome (CZS) and microcephaly. Early epilepsy surveillance is needed in children with prenatal ZIKV exposure; yet, most ZIKV-endemic regions do not have specialist epilepsy care. Here, we describe the demographic, clinical, imaging, and EEG characteristics of a 2-year-old child with CZS and microcephaly who presented with focal epileptiform activity, suboptimal growth, and severe neurodevelopmental delays. Administration of a brief seizure questionnaire by allied health professionals to the patient's caregiver helped to characterize the child's seizure semiology and differentiate focal from generalized seizure features. A telemedicine EEG interpretation platform provided valuable diagnostic information for the patient's local pediatrician to integrate into her treatment plan. This case illustrates that CZS can present with focal epilepsy features and that a telemedicine approach can be used to bridge the gap between epilepsy specialists and local care providers in resource limited ZIKV-endemic regions to achieve better seizure control in children with CZS.

Keywords: Congenital Zika Syndrome (CZS); Electroencephalography (EEG); Focal Epilepsy; Microcephaly; Telemedicine.

Fig. 1

Serial anthropometric assessment. Head circumference (HC), weight, and height were standardized for age using World Health Organization growth charts. Head circumference remains well below normal limits for age. Weight remains within normal limits at 2 and 15 months but declines to the lower end of the normal range by the age of 24 months. Length remains at the lower end of the normal range from 2 to 24 months.

Fig. 2

Magnetic resonance imaging (MRI) at 13 months (A–C). A: FLAIR axial view demonstrating enlarged lateral ventricles, predominantly in the right hemisphere. B: T1-weighted mid-sagittal view demonstrating intact corpus callosum and brain stem. C: T2-weighted coronal view demonstrating enlarged lateral ventricles and reduced cerebral volume, predominantly in the right hemisphere. D: Electroencephalography (EEG) at 16 months showing focal slowing (arrows) in left hemisphere.