Ketogenic diet in Dravet Syndrome: Tolerability and main benefits in children

Authors

DOI:

https://doi.org/10.33448/rsd-v13i10.47075

Keywords:

Myoclonics epilepsies, Ketogenic diet, Children.

Abstract

Dravet syndrome is a severe epileptic encephalopathy that manifests in early childhood and is characterized by hemiclonic or tonic-clonic seizures. One of the non-pharmacological treatment options for this condition is the ketogenic diet, which aims to induce ketosis in the body. This diet is widely recognized as a complementary therapy in the management of patients with epilepsy that is resistant to conventional medications. The present study aimed to evaluate the therapeutic role of the ketogenic diet in patients with Dravet syndrome, focusing on its efficacy as an adjunct therapy, diet tolerability, and the main reported adverse effects. For this, a bibliographic search was conducted in the PubMed database through a systematic literature review, analyzing publications from 2014 to 2024 in Portuguese and English. Based on the analyzed data, the use of the ketogenic diet proved to be a promising approach for the treatment of children with Dravet syndrome, demonstrating efficacy in reducing seizure frequency, with adverse effects considered acceptable and good tolerability.

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References

Aakash, M, Gautam, K, & Gulati, S. (2024). Advancements in Dravet Syndrome Therapeutics: A Comprehensive Look at Present and Future Treatment Horizons: A Focused Review. Annals of Indian Academy of Neurology. https://doi.org/10.4103/aian.aian_49_24

Anwar, A., Saleem, S., Patel, U. K., Arumaithurai, K., & Malik, P. (2019). Dravet Syndrome: An Overview. Cureus, 11(6). https://doi.org/10.7759/cureus.5006

Dyńka, D., Kowalcze, K., & Paziewska, A. (2022). The Role of Ketogenic Diet in the Treatment of Neurological Diseases. Nutrients, 14(23), 5003. https://doi.org/10.3390/nu14235003

Dressler, A., Trimmel-Schwahofer, P., Reithofer, E., Mühlebner, A., Gröppel, G., Reiter-Fink, E., Benninger, F., Grassl, R., & Feucht, M. (2015). Efficacy and tolerability of the ketogenic diet in Dravet syndrome - Comparison with various standard antiepileptic drug regimen. Epilepsy research, 109, 81–89. https://doi.org/10.1016/j.eplepsyres.2014.10.014

E. Wirrell C. (2016). Tratamento da Síndrome de Dravet. A revista canadense de ciências neurológicas. Le journal canadien des sciences neurologiques, 43 Suppl 3, S13–S18. https://doi.org/10.1017/cjn.2016.24

Kim, S. H., Kang, H. C., Lee, E. J., Lee, J. S., & Kim, H. D. (2017). Low glycemic index treatment in patients with drug-resistant epilepsy. Brain & development, 39(8), 687–692. https://doi.org/10.1016/j.braindev.2017.03.027

Knupp, K. G., Wirrel, E. C. (2018) Treatment strategies for Dravet Syndrome. CNS Drugs, 32 (4), 335-350. https://doi.org/10.1007/s40263-018-0511-y

Kortas, A., Schiller, K., Unterholzner, G., & Rauchenzauner, M. (2020). Accuracy of Flash Glucose Monitoring in a Patient with Dravet Syndrome on a Ketogenic Diet. Neuropediatrics, 51(1), 45–48. https://doi.org/10.1055/s-0039-1697621

Liu, F., Peng, J., Zhu, C., Xiao, H., He, F., Yin, F., & Chen, C. (2019). Efficacy of the ketogenic diet in Chinese children with Dravet syndrome: A focus on neuropsychological development. Epilepsy & behavior : E&B, 92, 98–102. https://doi.org/10.1016/j.yebeh.2018.12.016

Mahesan, A., Kamila, G., & Gulati, S. (2024). Advancements in Dravet syndrome therapeutics: A comprehensive look at present and future treatment horizons: A focused review. Annals of Indian Academy of Neurology, 27(4), 352-357. https://doi.org/10.4103/aian.aian_49_24

McGaugh, E., & Barthel, B. (2022). A Review of Ketogenic Diet and Lifestyle. Missouri medicine, 119(1), 84–88. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9312449/

Miller, I. O., & Sotero de Menezes, M. A. (2007) [Updated 2022]. SCN1A Seizure Disorders, GeneReviews. https://www.ncbi.nlm.nih.gov/books/NBK1318/

Pasca, L., De Giorgis, V., Macasaet, J. A., Trentani, C., Tagliabue, A., & Veggiotti, P. (2016). The changing face of dietary therapy for epilepsy. European journal of pediatrics, 175(10), 1267–1276. https://doi.org/10.1007/s00431-016-2765-z

Sampaio, L. P. de B. (2016). Ketogenic diet for epilepsy treatment. Arquivos de Neuro-Psiquiatria, 74(10), 842–848. https://doi.org/10.1590/0004-282x20160116

Scheffer, I. E., & Nabbout, R. (2019). SCN1A‐related phenotypes: Epilepsy and beyond. Epilepsia, 60(S3). https://doi.org/10.1111/epi.16386

Tian, X., Chen, J., Zhang, J., Yang, X., Ji, T., Zhang, Y., Wu, Y., Fang, F., Wu, X., & Zhang, Y. (2019). The Efficacy of Ketogenic Diet in 60 Chinese Patients With Dravet Syndrome. Frontiers in neurology, 10, 625. https://doi.org/10.3389/fneur.2019.00625

Wang, Y. Q., Fang, Z. X., Zhang, Y. W., Xie, L. L., e Jiang, L. (2020). Eficácia da dieta cetogênica em pacientes com síndrome de Dravet: Uma meta-análise. Seizure, 81, 36–42. https://doi.org/10.1016/j.seizure.2020.07.011

Wells, J., Swaminathan, A., Paseka, J., & Hanson, C. (2020). Efficacy and Safety of a Ketogenic Diet in Children and Adolescents with Refractory Epilepsy—A Review. Nutrients, 12(6), 1809. https://doi.org/10.3390/nu12061809

Wilmshurst, J. M., Burman, R., Gaillard, W. D., & Cross, J. H. (2015). Treatment of infants with epilepsy: Common practices around the world. Epilepsia, 56(7), 1033–1046. https://doi.org/10.1111/epi.13003

Wirrell, E. C. (2016). Treatment of Dravet Syndrome. Canadian Journal of Neurological Sciences / Journal Canadien Des Sciences Neurologiques, 43(S3), S13–S18. https://doi.org/10.1017/cjn.2016.249

Wu, Q., Wang, H., Fan, Y. Y., Zhang, J. M., Liu, X. Y., Fang, X. Y., Yang, F. H., Cao, Q. J., & Qi, Y. (2018). Ketogenic diet effects on 52 children with pharmacoresistant epileptic encephalopathy: A clinical prospective study. Brain and behavior, 8(5), e00973. https://doi.org/10.1002/brb3.973

Yan, N., Xin-Hua, W., Lin-Mei, Z., Yi-Ming, C., Wen-Hui, L., Yuan-Feng, Z., & Shui-Zhen, Z. (2018). Prospective study of the efficacy of a ketogenic diet in 20 patients with Dravet syndrome. Seizure, 60, 144–148. https://doi.org/10.1016/j.seizure.2018.06.023

Yu, M., Li, H., Sun, D., Li, D., Zhong, J., Gu, Q., Huang, S., Luo, R., Zhu, D., Yuan, B., Li, B., Xiao, N., Chen, Y., Zhang, Y., Wei, J., Jiang, Y., Liao, J., Qin, J., & China Association Against Epilepsy Ketogenic Diet Group (2023). The ketogenic diet for Dravet syndrome: A multicenter retrospective study. Nutrition (Burbank, Los Angeles County, Calif.), 110, 111976. https://doi.org/10.1016/j.nut.2023.111976

Zimeng, H., Li, Y., Zhao, X., Li, B. (2022). Dravet syndrome: Advances in etiology, clinical presentation, and treatment. Epilepsy Research, 108, 107041. https://doi.org/10.1016/j.eplepsyres.2022.107041

Published

2024-10-22

Issue

Section

Health Sciences

How to Cite

Ketogenic diet in Dravet Syndrome: Tolerability and main benefits in children. Research, Society and Development, [S. l.], v. 13, n. 10, p. e103131047075, 2024. DOI: 10.33448/rsd-v13i10.47075. Disponível em: https://ojs34.rsdjournal.org/index.php/rsd/article/view/47075. Acesso em: 28 jun. 2025.