Prognostic utility of hypsarrhythmia scoring in children with West syndrome after ketogenic diet

Abstract

ObjectiveThe aim of this study was to evaluate the clinical efficacy and electroencephalographic (EEG) changes of West syndrome after ketogenic diet (KD) therapy and to explore the correlation of EEG features and clinical efficacy. Patients and methodsWe retrospectively studied 39 patients with West syndrome who accepted KD therapy from May 2011 to October 2017. Outcomes including clinical efficacy and EEG features with hypsarrhythmia severity scores were analyzed. ResultsAfter 3 months of treatment, 20 patients (51.3%) had ≥50% seizure reduction, including 4 patients (10.3%) who became seizure-free. After 6 months of treatment, 4 patients remained seizure free, 12 (30.8%) had 90–99% seizure reduction, 8 (20.5%) had a reduction of 50–89%, and 15 (38.5%) had <50% reduction. Hypsarrhythmia scores were significantly decreased at 3 months of KD. They were associated with seizure outcomes at 6 months independent of gender, the course of disease and etiologies. Patients with a hypsarrhythmia score ≥8 at 3 months of therapy may not be benefited from KD. ConclusionOur findings suggest a potential benefit of KD for patients with drug-resistant West syndrome. Early change of EEG after KD may be a predictor of a patient’s response to the therapy.