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Abstract Details

Case Series: Congenital Zika Virus Infection associated with Epileptic Spasms
Infectious Disease
P4 - Poster Session 4 (5:30 PM-6:30 PM)
4-018
To describe four patients with congenital Zika Virus infection who developed intractable epileptic spasms (ES). 
Congenital Zika Virus (ZIKV) infection is unique to fetuses and infants infected with Zika virus before birth. This infection can result in a wide spectrum of structural and functional abnormalities; including, microcephaly, neuronal migrational disorders, hearing and vision abnormalities. The neuronal migration disorders usually lead to multiple neurological deficits, seizures and neurodevelopmental delay. However the complications of seizures and associated epileptic syndromes are poorly described in the literature. 
We describe a total of four patients with Congenital ZIKV and confirmed ZIKV exposure in utero who developed atypical epileptic spasms. 
Medical records were reviewed (n=4) and compatible with Congenital ZIKV exposure prenatally. All patients were evaluated at birth with Brain Magnetic Resonance Imaging (MRI) and electroencephalogram (EEG). All cases had normal EEG at birth with a variety of CNS dysgenesis including lissencephaly (75%), corpus callosum dysgenesis (100%), abnormal myelinization patterns (75%), and dysgenesis of brainstem (100%). Between 4 and 8 months of age, patients presented with flexor asymmetric spasms (50%) and extensor asymmetric spasms. At onset of epileptic spasms, an electroencephalogram was repeated and 100% were abnormal due to presence of epileptiform discharges. All patients were treated with high dose Methylprednisolone (20 mg/kg/day) with variable response. Fifty percent of patients required use of Vigabatrin due to refractory epileptic spasms and the other 50% remained with focal seizures requiring other anti-seizure medications. All patients persisted with intractable epilepsy and showed severe neurodevelopmental delay. 

Congenital ZIKV infection is highly associated with multiple neurological manifestations including central nervous system dysgenesis. We report a series of patients with associated intractable atypical ES unresponsive to treatment. There is limited data regarding pharmacologic response and long term neurological outcome in this patients, for which more studies are needed.

Authors/Disclosures
Mayela Marie Diaz Diaz, MD
PRESENTER
No disclosure on file
No disclosure on file
Janice Rodriguez Hernandez, MD (University of Puerto Rico, Child Neurology) No disclosure on file
Jessica Gonzalez Montes, MD No disclosure on file
Marisel Vazquez, MD (San Jorge Medical Building) Dr. Vazquez has nothing to disclose.
Mireya M. Bolo-Diaz, MD (Centro Pediatrico de Corozal) No disclosure on file
Jocelyn Montalvo, MD Dr. Montalvo has nothing to disclose.