Review
Nutrition & Dietetics
Urszula Skrobas, Piotr Duda, Lukasz Brylinski, Paulina Drozak, Magdalena Pelczar, Konrad Rejdak
Summary: Epilepsy is a global medical problem with a significant number of patients. Ketogenic dietary therapies have shown efficacy and safety in reducing seizures, especially in patients with Lennox-Gastaut syndrome (LGS).
Review
Behavioral Sciences
Kishan Vyas, Hannah Luedke, Benjamin Ruban-Fell
Summary: This review examines the content, availability, and development of treatment guidelines for the rare epileptic disorders Lennox-Gastaut syndrome, Dravet syndrome, CDKL5 deficiency disorder, tuberous sclerosis complex, and Rett syndrome in the Nordics region. The results show that most guidelines are specific to particular countries, with Sweden having the highest number of guidelines and no guidelines identified for Iceland. The development methodologies of the guidelines also vary, and some do not report details on the evidence sources used. The availability of treatment recommendations differs across disorders.
BRAIN AND BEHAVIOR
(2022)
Review
Clinical Neurology
Cecilie Johannessen Landmark, Heidrun Potschka, Stephane Auvin, Jo M. Wilmshurst, Svein Johannessen, Dorothee Kasteleijn-Nolst Trenite, Elaine C. Wirrell
Summary: DEEs, characterized by frequent and severe seizures and pharmacoresistance, have seen the development of new treatment options in recent years, including cannabidiol, everolimus, and repurposed drugs like fenfluramine, offering hope for improved management of patients with DEEs.
Article
Behavioral Sciences
Jurriaan M. Peters, Vinay Puri, Eric Segal, Sunita N. Misra, Adrian L. Rabinowicz, Enrique Carrazana
Summary: People with epilepsy often experience frequent seizure activity, and benzodiazepines are the main form of treatment. Cannabidiol (CBD) can be used as an additional treatment for epilepsy, and it may interact with benzodiazepines. This study examines the safety and effectiveness of using diazepam nasal spray in patients with seizure clusters who also use CBD. The results suggest that CBD does not affect the safety and effectiveness of diazepam nasal spray, and it can be used alongside in appropriate patients.
EPILEPSY & BEHAVIOR
(2023)
Article
Clinical Neurology
Camilo Espinosa-Jovel, Sandra Riveros, Carlos Bolanos-Almeida, Mateo Ramirez Salazar, Leidy Ceballos Inga, Laura Guio
Summary: The study demonstrates that highly purified CBD is safe and effective in treating drug resistant epilepsy, regardless of etiology or syndrome. Further controlled trials are needed to validate this finding.
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY
(2023)
Article
Clinical Neurology
Luigi Francesco Iannone, Gabriele Arena, Domenica Battaglia, Francesca Bisulli, Paolo Bonanni, Antonella Boni, Maria Paola Canevini, Gaetano Cantalupo, Elisabetta Cesaroni, Manuela Contin, Antonietta Coppola, Duccio Maria Cordelli, Giovanni Cricchiuti, Valentina De Giorgis, Maria Fulvia De Leva, Marta De Rinaldis, Giuseppe d'Orsi, Maurizio Elia, Carlo Andrea Galimberti, Alessandra Morano, Tiziana Granata, Renzo Guerrini, Monica A. M. Lodi, Angela La Neve, Francesca Marchese, Silvia Masnada, Roberto Michelucci, Margherita Nosadini, Nicola Pilolli, Dario Pruna, Francesca Ragona, Anna Rosati, Margherita Santucci, Alberto Spalice, Nicola Pietrafusa, Pasquale Striano, Elena Tartara, Laura Tassi, Amanda Papa, Claudio Zucca, Emilio Russo, Oriano Mecarelli
Summary: In this study, purified cannabidiol (CBD) was administered to highly refractory patients with Dravet (DS) or Lennox-Gastaut (LGS) syndromes in an ongoing expanded access program. The interim results showed that CBD is associated with improved seizure control in a considerable proportion of patients, and its safety and effectiveness are not dose-related.
FRONTIERS IN NEUROLOGY
(2021)
Article
Medicine, General & Internal
Antonio Silvinato, Idevaldo Floriano, Wanderley Marques Bernardo
Summary: This systematic review with meta-analysis evaluates the efficacy, safety, and tolerability of CBD as an adjunct treatment for seizures in patients with Dravet syndrome, Lennox-Gataut syndrome, or tuberous sclerosis complex. The results show that CBD significantly reduces the frequency of seizures, increases the number of patients with a positive response to the treatment, and has acceptable toxicity.
REVISTA DA ASSOCIACAO MEDICA BRASILEIRA
(2022)
Review
Clinical Neurology
Simona Lattanzi, Gaetano Zaccara, Emilio Russo, Angela La Neve, Monica Anna Maria Lodi, Pasquale Striano
Summary: Pharmaceutically purified oral cannabidiol (CBD) has been approved for treating seizures associated with Dravet syndrome (DS) and Lennox-Gastaut syndrome (LGS), showing efficacy in managing symptoms such as somnolence, gastrointestinal discomfort, and increase in serum transaminases. CBD's metabolism in the liver can influence drug metabolism enzymes, particularly in interactions with antiseizure medications like clobazam. The findings pertain specifically to high-grade purified CBD oral solution and may not be applicable to other cannabis-based products.
EXPERT REVIEW OF NEUROTHERAPEUTICS
(2021)
Review
Clinical Neurology
Richard F. Chin, Ana Mingorance, Benjamin Ruban-Fell, Isabelle Newell, Jenni Evans, Kishan Vyas, Charlotte Nortvedt, Sam Amin
Summary: This review analyzed country-specific and international treatment guidelines for DS, LGS, and CDD, finding a lack of treatment guidelines for CDD and variability in the development process and treatment recommendations for DS and LGS. Supra-national consensus guidance based on wider geographical data may improve care standards.
FRONTIERS IN NEUROLOGY
(2021)
Article
Nutrition & Dietetics
Mei Yu, Hua Li, Dan Sun, Dan Li, Jianmin Zhong, Qiang Gu, Shaoping Huang, Rong Luo, Dengna Zhu, Baoqiang Yuan, Baomin Li, Nong Xiao, Yucai Chen, Yuqin Zhang, Jurong Wei, Yuwu Jiang, Jianxiang Liao, Jiong Qin
Summary: This study is a retrospective multicenter cohort study from 14 centers in China, analyzing the efficacy and safety of the ketogenic diet (KD) for the treatment of Dravet syndrome (DS) based on a large number of cases. The results showed that KD is effective for patients with DS and has a low incidence of side effects.
Review
Pharmacology & Pharmacy
James E. Frampton
Summary: Fintepla is an oral anti-seizure medication with a novel mechanism of action. It has been shown to reduce seizure frequency in patients with Dravet syndrome and Lennox-Gastaut syndrome, and may also improve everyday executive functioning in some patients.
Review
Clinical Neurology
Rima Nabbout, Alexis Arzimanoglou, Stephane Auvin, Patrick Berquin, Archana Desurkar, Douglas Fuller, Charlotte Nortvedt, Patrizia Pulitano, Anna Rosati, Victor Soto, Vicente Villanueva, J. Helen Cross
Summary: This retrospective chart review study evaluated the use of plant-derived highly purified cannabidiol (CBD) as add-on therapy in patients with Lennox-Gastaut syndrome (LGS) or Dravet syndrome (DS). The results showed that CBD had a positive impact on seizure frequency in some patients, but there were also adverse events reported.
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY
(2023)
Article
Genetics & Heredity
Justyna Paprocka, Szymon Zietkiewicz, Joanna Kosinska, Ewa Kaczorowska, Rafal Ploski
Summary: SH3 and multiple ankyrin repeat domains (SHANKs) are essential scaffolding proteins in excitatory synapses. While SHANK3 defects are known to cause neurodevelopmental disorders, including autism and epilepsy, less is known about the disease associations of SHANK1. A novel variant in SHANK1 was identified as the monogenic cause of intellectual disability, autism, and refractory epilepsy in a patient, with significant improvement seen in epilepsy with cannabidiol therapy. Computational analyses revealed the deleterious effect of the SHANK1 variant on protein structure, disrupting a conserved region and potentially impacting protein-protein interactions.
FRONTIERS IN GENETICS
(2021)
Article
Clinical Neurology
Vicino Walter, Muccioli Lorenzo, Pondrelli Federica, Licchetta Laura, Stipa Carlotta, Mostacci Barbara, Di Vito Lidia, Ferri Lorenzo, Cancellerini Chiara, Solda Martina, Tinuper Paolo, Bisulli Francesca
Summary: This study evaluated the efficacy and safety of cannabidiol (CBD) for the treatment of epilepsy in a real-world setting. The results showed that CBD was a safe and effective therapeutic option for highly drug-resistant patients, leading to a significant reduction in seizure frequency in over one-fourth of them.
SEIZURE-EUROPEAN JOURNAL OF EPILEPSY
(2023)
Article
Pharmacology & Pharmacy
Manuela Contin, Susan Mohamed, Margherita Santucci, Monica Anna Maria Lodi, Emilio Russo, Oriano Mecarelli
Summary: This study explored the clinical pharmacokinetics of cannabidiol (CBD) in patients with Dravet and Lennox-Gastaut syndrome, revealing higher CBD C/D in adults and the potential need for reduced weight-normalized doses with aging to achieve the same CBD plasma levels.
FRONTIERS IN PHARMACOLOGY
(2021)
Article
Behavioral Sciences
Kylie Holden, Adam L. Hartman
EPILEPSY & BEHAVIOR
(2018)
Article
Clinical Neurology
Alison Dolce, Polan Santos, Weiran Chen, Ahmet Hoke, Adam L. Hartman
Article
Critical Care Medicine
Ajit Sarnaik, Nikki Miller Ferguson, A. M. Iqbal O'Meara, Shruti Agrawal, Akash Deep, Sandra Buttram, Michael J. Bell, Stephen R. Wisniewski, James F. Luther, Adam L. Hartman, Monica S. Vavilala
NEUROCRITICAL CARE
(2018)
Article
Clinical Neurology
Kathryn C. Fitzgerald, Diane Vizthum, Bobbie Henry-Barron, Amy Schweitzer, Sandra D. Cassard, Eric Kossoff, Adam L. Hartman, Dimitrios Kapogiannis, Patrick Sullivan, David J. Baer, Mark P. Mattson, Lawrence J. Appel, Ellen M. Mowry
MULTIPLE SCLEROSIS AND RELATED DISORDERS
(2018)
Article
Clinical Neurology
Kyle A. Metz, Xinchen Teng, Isabelle Coppens, Heather M. Lamb, Bart E. Wagner, Jill A. Rosenfeld, Xianghui Chen, Yu Zhang, Hee Jong Kim, Michael E. Meadow, Tim Sen Wang, Edda D. Haberlandt, Glenn W. Anderson, Esther Leshinsky-Silver, Weimin Bi, Thomas C. Markello, Marsha Pratt, Nawal Makhseed, Adolfo Garnica, Noelle R. Danylchuk, Thomas A. Burrow, Parul Jayakar, Dianalee McKnight, Satish Agadi, Hatha Gbedawo, Christine Stanley, Michael Alber, Isabelle Prehl, Katrina Peariso, Min Tsui Ong, Santosh R. Mordekar, Michael J. Parker, Daniel Crooks, Pankaj B. Agrawal, Gerard T. Berry, Tobias Loddenkemper, Yaping Yang, Gustavo H. B. Maegawa, Abdel Aouacheria, Janet G. Markle, James A. Wohlschlegel, Adam L. Hartman, J. Marie Hardwick
ANNALS OF NEUROLOGY
(2018)
Editorial Material
Biotechnology & Applied Microbiology
Mustafa Sahin, Stephanie R. Jones, John A. Sweeney, Elizabeth Berry-Kravis, Barry W. Connors, Joshua B. Ewen, Adam L. Hartman, April R. Levin, William Z. Potter, Laura A. Mamounas
NATURE REVIEWS DRUG DISCOVERY
(2019)
Article
Biochemistry & Molecular Biology
Adam L. Hartman, Anneliene Hechtelt Jonker, Melissa A. Parisi, Daria Julkowska, Nicole Lockhart, Rosario Isasi
EUROPEAN JOURNAL OF HUMAN GENETICS
(2020)
Article
Clinical Neurology
A. Berrington, K. C. Schreck, B. J. Barron, L. Blair, D. D. M. Lin, A. L. Hartman, E. Kossoff, L. Easter, C. T. Whitlow, Y. Jung, F-C Hsu, M. C. Cervenka, J. O. Blakeley, P. B. Barker, R. E. Strowd
AMERICAN JOURNAL OF NEURORADIOLOGY
(2019)
Editorial Material
Clinical Neurology
Adam L. Hartman
Article
Clinical Neurology
Mackenzie Cervenka, Juan M. Pascual, Jong M. Rho, Elizabeth Thiele, Gary Yellen, Vicky Whittemore, Adam L. Hartman
Summary: Despite past efforts to evaluate metabolism-based therapies for epilepsy, there is still uncertainty on how alterations in metabolism relate to epilepsy. A comprehensive, creative, and inclusive problem-directed research agenda is needed, with renewed adherence to rigor and transparency across all investigation levels, to benefit people with epilepsy.
ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY
(2021)
Editorial Material
Clinical Neurology
Adam L. Hartman
Article
Clinical Neurology
Jacqueline A. French, Martina Bebin, Marc A. Dichter, Jerome Engel, Adam L. Hartman, Sergiusz Jozwiak, Pavel Klein, James McNamara, Roy Twyman, Paul Vespa
Summary: The 2018 NINDS workshop focused on optimizing and accelerating the development of therapies for antiepileptogenesis and disease modification in the epilepsies, addressing nomenclature, trial design complexities, drug dosing, and regulatory hurdles.
Meeting Abstract
Clinical Neurology
Karisa Schreck, Adam Berrington, Bobbie Henry-Barron, Lindsay Blair, Adam Hartman, Eric Kossoff, Linda Easter, Chris Whitlow, Fang-Chi Hsu, Mackenzie Cervenka, Jaishri Blakeley, Peter Barker, Roy Strowd
Article
Critical Care Medicine
Nikki Miller Ferguson, Ajit Sarnaik, Darryl Miles, Nadeem Shafi, Mark J. Peters, Edward Truemper, Monica S. Vavilala, Michael J. Bell, Stephen R. Wisniewski, James F. Luther, Adam L. Hartman, Patrick M. Kochanek
CRITICAL CARE MEDICINE
(2017)
Article
Biochemistry & Molecular Biology
Choyce A. Weatherly, Siqi Du, Curran Parpia, Polan T. Santos, Adam L. Hartman, Daniel W. Armstrong
ACS CHEMICAL NEUROSCIENCE
(2017)