Therapeutic activity of cannabidiol in the treatment of Dravet syndrome
DOI:
https://doi.org/10.33448/rsd-v12i5.41792Keywords:
Dravet Syndrome; Cannabidiol; Effectiveness; Treatment.Abstract
The Dravet syndrome (DS) is a genetically rare disease, also known as severe myoclonic epilepsy of childhood, associated with motor and behavioral comorbidities such as hyperactivity, sleep disturbance, deficits in social interaction and sudden death. There is a need for therapeutic approaches that control Dravet syndrome, as available conventional antiepileptic drugs have low efficacy. Therefore, the present work aimed to describe the therapeutic potential of cannabidiol to treat Dravet syndrome. Cannabidiol is a substance derived from the plant cannabis sativa that acts on the central nervous system and has been gaining ground in medicine to treat different diseases such as disorders in muscle control, tics, neuropathic pain, tuberous sclerosis, epileptic encephalopathy, among other pathologies. According to the studies cited in this work, cannabidiol to treat Dravet syndrome has demonstrated effectiveness from doses of 20mg/kg/day and CBD has shown efficacy when used in the long term. In addition to the decrease in seizures, comorbidities associated with the pathology were also reduced after cannabidiol administration. This work is an integrative review, the databases used were PubMed and ScienceDirect. 5 PubMed articles and 1 ScienceDirect article were included in the final sample. The descriptors were used: Epilepsies Myoclonic and Cannabidiol in Portuguese, English and Spanish that were combined with the Boolean operator “AND”. It is concluded that cannabidiol administered with appropriate dosages is effective to treat severe childhood epilepsy, including seizures and comorbidities associated with pathology in different age groups.
References
Anderson, L. L., Absalom, N. L., Abelev, S. V., Low, I. K., Doohan, P. T., Martin, L. J., & Arnold, J. C. (2019). Coadministered cannabidiol and clobazam: Preclinical evidence for both pharmacodynamic and pharmacokinetic interactions. Epilepsia, 60(11), 2224-2234.
Bardin L. (1977). Análise de conteúdo. Lisboa: Edições, 70.
Caraballo, R., Reyes, G., Demirdjian, G., Huaman, M., & Gutierrez, R. (2022). Long-term use of cannabidiol-enriched medical cannabis in a prospective cohort of children with drug-resistant developmental and epileptic encephalopathy. Seizure, 95, 56-63.
Davis, B. H., Beasley, T. M., Amaral, M., Szaflarski, J. P., Gaston, T., Perry Grayson, L., & UAB CBD Study Group (includes all the investigators involved in the UAB EAP CBD program). (2021). Pharmacogenetic predictors of cannabidiol response and tolerability in treatment‐resistant epilepsy. Clinical Pharmacology & Therapeutics, 110(5), 1368-1380.
Devinsky, O., Cross, J. H., Laux, L., Marsh, E., Miller, I., Nabbout, R., & Wright, S. (2017). Trial of cannabidiol for drug-resistant seizures in the Dravet syndrome. New England Journal of Medicine, 376(21), 2011-2020.
Devinsky, O., Nabbout, R., Miller, I., Laux, L., Zolnowska, M., Wright, S., & Roberts, C. (2019). Long‐term cannabidiol treatment in patients with Dravet syndrome: An open‐label extension trial. Epilepsia, 60(2), 294-302.
Devinsky, O., Patel, A. D., Thiele, E. A., Wong, M. H., Appleton, R., Harden, C. L., & GWPCARE1 Part A Study Group. (2018). Randomized, dose-ranging safety trial of cannabidiol in Dravet syndrome. Neurology, 90(14), e1204-e1211.
D'Onofrio, G., Kuchenbuch, M., Camus, H. L., Desnous, B., Staath, V., Napuri, S., & Nabbout, R. (2020). Slow titration of cannabidiol add-on in drug-resistant epilepsies can improve safety with maintained efficacy in an open-label study. Frontiers in Neurology, 829.
Gil-Nagel, A., Sánchez-Carpintero, R., & Villanueva, V. (2023). Patient profile, management, and quality of life associated with Dravet syndrome: a cross-sectional, multicentre study of 80 patients in Spain. Scientific Reports, 13(1), 3355.
Huang, C. H., Hung, P. L., Fan, P. C., Lin, K. L., Hsu, T. R., Chou, I. J., & Lee, W. T. (2021). Clinical spectrum and the comorbidities of Dravet syndrome in Taiwan and the possible molecular mechanisms. Scientific reports, 11(1), 20242.
Lagae, L., Brambilla, I., Mingorance, A., Gibson, E., & Battersby, A. (2018). Quality of life and comorbidities associated with Dravet syndrome severity: a multinational cohort survey. Developmental Medicine & Child Neurology, 60(1), 63-72.
Lagae, L., Sullivan, J., Knupp, K., Laux, L., Polster, T., Nikanorova, M., & Ceulemans, B. (2019). Fenfluramine hydrochloride for the treatment of seizures in Dravet syndrome: a randomised, double-blind, placebo-controlled trial. The Lancet, 394(10216), 2243-2254.
Lai, W. W., Galer, B. S., Wong, P. C., Farfel, G., Pringsheim, M., Keane, M. G., & Agarwal, A. (2020). Cardiovascular safety of fenfluramine in the treatment of Dravet syndrome: analysis of an ongoing long‐term open‐label safety extension study. Epilepsia, 61(11), 2386-2395.
Laux, L. C., Bebin, E. M., Checketts, D., Chez, M., Flamini, R., Marsh, E. D., & CBD EAP study group. (2019). Long-term safety and efficacy of cannabidiol in children and adults with treatment resistant Lennox-Gastaut syndrome or Dravet syndrome: Expanded access program results. Epilepsy research, 154, 13-20.
Madan Cohen, J., Checketts, D., Dunayevich, E., Gunning, B., Hyslop, A., Madhavan, D., & Zuberi, S. M. (2021). Time to onset of cannabidiol treatment effects in Dravet syndrome: analysis from two randomized controlled trials. Epilepsia, 62(9), 2218-2227.
Patra, P. H., Serafeimidou‐Pouliou, E., Bazelot, M., Whalley, B. J., Williams, C. M., & McNeish, A. J. (2020). Cannabidiol improves survival and behavioural co‐morbidities of Dravet syndrome in mice. British Journal of Pharmacology, 177(12), 2779-2792.
Scheffer, I. E., Halford, J. J., Miller, I., Nabbout, R., Sanchez‐Carpintero, R., Shiloh‐Malawsky, Y., & Devinsky, O. (2021). Add‐on cannabidiol in patients with Dravet syndrome: Results of a long‐term open‐label extension trial. Epilepsia, 62(10), 2505-2517.
Shmuely, S., Surges, R., Helling, R. M., Gunning, W. B., Brilstra, E. H., Verhoeven, J. S., & Thijs, R. D. (2020). Cardiac arrhythmias in Dravet syndrome: an observational multicenter study. Annals of clinical and translational neurology, 7(4), 462-473.
Souza, M. T. D., Silva, M. D. D., & Carvalho, R. D. (2010). Revisão integrativa: o que é? Como fazer isso? Einstein (São Paulo), 8, 102-106.
Sun, Y., & Dolmetsch, R. E. (2018, January). Investigating the therapeutic mechanism of cannabidiol in a human induced pluripotent stem cell (iPSC)-based model of Dravet syndrome. In Cold Spring Harbor Symposia on Quantitative Biology (Vol. 83, pp. 185-191). Cold Spring Harbor Laboratory Press.
Wheless, J. W., Dlugos, D., Miller, I., Oh, D. A., Parikh, N., Phillips, S., & INS011-14-029 Study Investigators. (2019). Pharmacokinetics and tolerability of multiple doses of pharmaceutical-grade synthetic cannabidiol in pediatric patients with treatment-resistant epilepsy. CNS drugs, 33, 593-604.
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