Ketogenic Diet or Vagal Nerve Stimulation in Children with Tuberous Sclerosis Complex and Drug-Refractory Epilepsy
Abstract number :
1.222
Submission category :
4. Clinical Epilepsy / 4C. Clinical Treatments
Year :
2021
Submission ID :
1826484
Source :
www.aesnet.org
Presentation date :
12/4/2021 12:00:00 PM
Published date :
Nov 22, 2021, 06:54 AM
Authors :
Kaajal Parbhoo, MBBCh, FCPaed(SA), Cert Paed Neurology(SA) - Nelson Mandela Children's Hospital; Ahmed Abushama - Epilepsy Fellow, Division of Neurology, Department of Paediatrics, The Hospital for Sick Children; Robyn Whitney - Paediatric Epileptologist/Neurologist, Comprehensive Paediatric Epilepsy Program, Division of Neurology, Department of Paediatrics, McMaster University; Hassan Kiani - Undergraduate Student, McMaster University; Adrian Salopek - Undergraduate Student, McMaster University; Rajesh RamachandranNair - Paediatric Epileptologist/Neurologist, Comprehensive Paediatric Epilepsy Program, Division of Neurology, Department of Paediatrics, McMaster University; Kevin Jones - Paediatric Epileptologist/Neurologist, Comprehensive Paediatric Epilepsy Program, Division of Neurology, Department of Paediatrics, McMaster University; Maria Zak - Nurse Practitioner, Epilepsy Program, Division of Neurology, Department of Paediatrics, The Hospital for Sick Children; Ivanna Yau - Nurse Practitioner, Epilepsy Program, Division of Neurology, Department of Paediatrics, The Hospital for Sick Children; Elizabeth Donner - Paediatric Epileptologist/Neurologist, Epilepsy Program, Division of Neurology, Department of Paediatrics, The Hospital for Sick Children; Cristina Go - Paediatric Epileptologist/Neurologist, Epilepsy Program, Division of Neurology, Department of Paediatrics, The Hospital for Sick Children; Puneet Jain - Paediatric Epileptologist/Neurologist, Epilepsy Program, Division of Neurology, Department of Paediatrics, The Hospital for Sick Children
Rationale: Tuberous sclerosis complex (TSC) is often associated with early onset, drug refractory epilepsy (DRE). The ketogenic diet (KD) and vagal nerve stimulation (VNS) are non-pharmacological therapies that are commonly used in patients with TSC who are not candidates for resective surgery. There are only a small number of studies looking at the effectiveness of dietary therapies and VNS in TSC. The aim of this study was to determine the efficacy of dietary therapies and VNS in DRE associated with TSC.
Methods: A retrospective chart review was conducted which included children with TSC and DRE who were not candidates for epilepsy surgery and who were treated with dietary therapy or had VNS therapy, at two pediatric academic centers in Ontario, Canada from 2010 to 2020. Seizure outcomes and adverse effects were assessed.
Results: Twenty children were included, the median age at seizure onset was 5 months (range 2 days - 2.5 years). All children had daily seizures at initiation of dietary therapy and at VNS implantation; the most common seizure type was focal seizures. TSC2 variants were identified in the majority, 11/15 (73%) children. One or more TSC associated neuropsychiatric disorder (TAND) features were identified in all children. Fifteen children (75%) were treated with dietary therapies (10 classic ketogenic diet, 4 MCT diet and 1 modified Atkins diet) with median age at initiation 4.2 years (IQR 2.5-11.7); 10/15 (67%) had persistently good ketone levels. Seven out of 15 children (47%) had >50% reduction in seizure frequency after a median follow-up duration of 23 months on dietary therapy. Eleven discontinued the diet (3–side effects, 3–not effective, 2–improved seizure control, 3-other) after a median of 17 months (range 1-108 months). The most common dietary adverse effects were constipation in 47% (7/15) and vomiting in 33% (5/15). Five of the 9 children who had VNS, were referred for VNS after failed or discontinued dietary therapy. Median age at implantation was 12 years; 6/9 (67%) had >50% reduction in seizures after a median follow up of 37 months; 7/9 (78%) had some response to the magnet (4-aborted seizures, 2-reduced seizure duration, 1-reduced seizure severity). VNS therapy was generally well tolerated.
Conclusions: In this cohort of children with TSC and DRE, nearly half of the children had >50% seizure reduction on dietary therapies; nearly two-thirds of the children had >50% seizure reduction after VNS implantation. A systematic review and meta-analysis of the existing small studies may be warranted to further assess the effect of these therapies in children with TSC.
Funding: Please list any funding that was received in support of this abstract.: Nil.
Clinical Epilepsy