The Efficacy and Tolerability of the Ketogenic Diet in Treatment of Epileptic Spasms after Failure of Vigabatrin and/or Steroids
Abstract number :
2.201
Submission category :
4. Clinical Epilepsy / 4C. Clinical Treatments
Year :
2017
Submission ID :
349543
Source :
www.aesnet.org
Presentation date :
12/3/2017 3:07:12 PM
Published date :
Nov 20, 2017, 11:02 AM
Authors :
Qi Xu, BC Children's Hospital, University of British Columbia; Anita Datta, BC Children's Hospital, University of British Columbia; Aspasia Michoulas, BC Children's Hospital, University of BC; Alex xu, BC Children's Hospital, University of British Columbi
Rationale: To evaluate the efficacy and tolerability of the early use of the ketogenic diet (KD) in infants with Epileptic Spasms (ES) who are refractory to Vigabatrin and/or steroids (ACTH/oral prednisolone). Methods: This is a retrospective study of all infants with ES who received the KD at our hospital between 2014 to 2016. All infants with ES with severe epileptic encephalopathy refractory to vigabatrin and/or steroids were treated with the KD. The information of efficacy and side effects of KD were obtained. Secondary outcomes of EEG results and neurodevelopment status were collected. The cessation of ES was defined as the absence of clinical spasms from within 14 days of commencement of treatment while electroclinical cessation of ES with resolution of hypsarrhythmia. Results: A total 9 infants who had epileptic encephalopathy and spasms were included in this study (7 males). The mean age of at onset of ES was 5.6 months (95% CI 2.3-8.9), and mean age at initiation of KD was 8.9 months (95% CI 5.8-11.9). Seven cases had West syndrome and two had overlapping Ohtahara syndrome and West syndrome. The etiology of ES infants included prematurity with periventricular leukomalacia, hemorrhagic stroke, tuberous sclerosis, cortical dysplasia, trisomy 21, ARX mutation, STXBP1 mutation, adenylosuccinate lyase deficiency and one unknown. All of these infants had epileptic spasms clinically and EEG initially showed hypsarrhythmia.Among these 9 infants, 3 (33%) had cessation of ES and hypsarrhythmia, 1 (11%) case had ES cessation for 3 months and then recurrence with improvement of hypsarrhythmia. In other cases, 1 case had >50% reduction of spasm with EEG improvement, 2 had >50% reduction of spasm but no EEG improvement, 1 had no response and 1 had deterioration. In terms of the infants with the Ohtahara syndrome, one had >50% reduction, and one had deterioration when they were on KD treatment. Nine infants tolerated the KD well and one cases experienced feeding intolerance and parental challenges after 6 months of KD. One infant died at 16 months of age. (Table 1 for the details of all infants) Conclusions: Ketogenic diet is a safe and potentially effective treatment for infants with epileptic spasms after steroid and vigabatrin therapy. Our findings are consistent with previous studies. Funding: Rare Disease Foundation, microgrant
Clinical Epilepsy