Clinical characteristics and treatment outcomes of pediatric patients with postencephalitic epilepsy characterized by epileptic spasms

Abstract

PurposeViral encephalitis (VE) or bacterial meningoencephalitis (BME) in early childhood may cause brain injury and neurological sequelae, including epilepsy. Postencephalitic epilepsy (PEE) characterized by epileptic spasms (ES) is a rare but serious condition; there is an urgent need to develop new methods to evaluate the characteristics of these children and select appropriate treatments. MethodsWe conducted an observational study of 20 patients (11 males, 9 females) who experienced ES after VE or BME at the Chinese PLA General Hospital. Patients were followed up for over 12 months, and outcomes were analyzed. ResultsThe median ages at the onset of encephalitis and ES were 5.5 and 11.5 months, respectively. The median age at follow-up was 35.5 months. Sixteen (80 %) patients developed drug-resistant epilepsy (DRE), including all 12 patients with VE and 4 of 8 patients with BME. Epileptiform discharges were detected on electroencephalography, including 15 patients with hypsarrhythmia and 5 without. Fifteen of the patients were treated with a 14-day intravenous infusion of adrenocorticotropic hormone (ACTH) at a dose 2.5 U/kg (≤25 U); 12 showed a short-term response but 10 experienced recurrence. Three patients received vigabatrin, and none of these patients responded to treatment. Six patients started a ketogenic diet (KD); five failed to respond and the outcome was not known in one. Four patients were treated by vagus nerve stimulation (VNS), and all showed a partial response. ConclusionChildren with PEE characterized by ES are more likely to develop DRE. The prognosis was worse for patients with VE compared to those with BME. Clarifying the efficacies of treatments involving ACTH (low-dose), KD, vigabatrin, and VNS will require further investigation.