Epileptic spasms as the presenting seizure type in a patient with a new "O" of TORCH, congenital Zika virus infection

Abstract

Congenital TORCH infections are a significant cause of epileptic spasms, an infantile epileptic encephalopathy, through disruptions to several pathways in neurodevelopment. Congenital Zika virus has a similar neurotropism to other TORCH agents, and leads to microcephaly, severe neurodevelopmental impairment, and high rates of early onset seizures. Here we report a child with confirmed congenital Zika virus who developed extensor epileptic spasms and hypsarrhythmia associated with a loss of early developmental milestones. Early treatment led to resolution of epileptic spasms and improved developmental trajectory, though the child continues to have ongoing focal seizures and prominent developmental impairment. Congenital Zika virus infection requires close monitoring as early identification of epileptic spasms is likely important in long term developmental outcome.

Fig. 1

(A,B) Cranial ultrasound at 1 week. A: parasagittal and B: coronal views through the anterior fontanelle demonstrating extensive punctate calcifications in the periventricular and subcortical white matter. (C–E) C: Sagittal MPR image demonstrating small cranium relative to face, and small supratentorial structures relative to brainstem and cerebellum. Axial (D) and coronal (E) T2-weighted images showing small cerebral hemispheres with reduced white matter volume, ex vacuo dilation of lateral ventricles, and extensive bilateral polymicrogyria most prominent in the anterior and superior frontal lobes.

Fig. 2

(A,B) EEG at 4 months of age, bipolar montage. A: Background activity in sleep displays multifocal spikes with most frequent discharges over the temporal regions. Background activity abnormal with high amplitude slow waves reaching 300 μV over the right hemisphere and lack of normal features of sleep. B: Clinical spasms seen in the awake state (arrow) with a high amplitude slow wave maximally over the right temporal region followed by diffuse attenuation. The background is disorganized with prominent delta slowing. (C) EEG at 16 months of age in sleep shows resolution of background encephalopathy but epileptiform activity over the right parietal region (P8).