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2. Comparative Effectiveness of Initial Treatment for Infantile Spasms in a Contemporary US Cohort

Author

Grinspan, Zachary M, Knupp, Kelly G, Patel, Anup D, Yozawitz, Elissa G, Wusthoff, Courtney J, Wirrell, Elaine C, Valencia, Ignacio, Singhal, Nilika S, Nordli, Douglas R, Mytinger, John R, Mitchell, Wendy G, Keator, Cynthia G, Loddenkemper, Tobias, Hussain, Shaun A, Harini, Chellamani, Gaillard, William D, Fernandez, Ivan S, Coryell, Jason, Chu, Catherine J, Berg, Anne T, and Shellhaas, Renee A

Subjects
Biomedical and Clinical Sciences, Clinical Sciences, Pediatric, Comparative Effectiveness Research, Brain Disorders, Neurosciences, Cognitive Sciences, Neurology & Neurosurgery, Clinical sciences
Abstract

ObjectiveTo compare the effectiveness of initial treatment for infantile spasms.MethodsThe National Infantile Spasms Consortium prospectively followed up children with new-onset infantile spasms that began at age 2 to 24 months at 23 US centers (2012-2018). Freedom from treatment failure at 60 days required no second treatment for infantile spasms and no clinical spasms after 30 days of treatment initiation. We managed treatment selection bias with propensity score weighting and within-center correlation with generalized estimating equations.ResultsFreedom from treatment failure rates were as follows: adrenocorticotropic hormone (ACTH) 88 of 190 (46%), oral steroids 42 of 95 (44%), vigabatrin 32 of 87 (37%), and nonstandard therapy 4 of 51 (8%). Changing from oral steroids to ACTH was not estimated to affect response (observed 44% estimated to change to 44% [95% confidence interval 34%-54%]). Changing from nonstandard therapy to ACTH would improve response from 8% to 39% (17%-67%), and changing to oral steroids would improve response from 8% to 38% (15%-68%). There were large but not statistically significant estimated effects of changing from vigabatrin to ACTH (29% to 42% [15%-75%]), from vigabatrin to oral steroids (29% to 42% [28%-57%]), and from nonstandard therapy to vigabatrin (8% to 20% [6%-50%]). Among children treated with vigabatrin, those with tuberous sclerosis complex (TSC) responded more often than others (62% vs 29%; p < 0.05).DiscussionCompared to nonstandard therapy, ACTH and oral steroids are superior for initial treatment of infantile spasms. The estimated effectiveness of vigabatrin is between that of ACTH/oral steroids and nonstandard therapy, although the sample was underpowered for statistical confidence. When used, vigabatrin worked best for TSC.Classification of evidenceThis study provides Class III evidence that for children with new-onset infantile spasms, ACTH or oral steroids were superior to nonstandard therapies.

Published
2021

7. Safety and efficacy of ganaxolone in patients with CDKL5 deficiency disorder: results from the double-blind phase of a randomised, placebo-controlled, phase 3 trial

Author

Belousova, Elena, Belyaev, Oleg, Ben-Zeev, Bruria, Brunklaus, Andreas, Ciliberto, Michael A., Darra, Francesca, Davis, Ronald, De Giorgis, Valentina, Doronina, Olga, Fahey, Michael, Guerrini, Renzo, Heydemann, Peter, Khaletskaya, Olga, Lisewski, Pawel, Marsh, Eric D., Moosa, Ahsan N., Perry, Scott, Philip, Sunny, Rajaraman, Rajsekar R., Renfroe, Ben, Saneto, Russell P., Scheffer, Ingrid E., Sogawa, Yoshimi, Suter, Bernhardt, Sweney, Matthew T., Tarquinio, Daniel, Veggiotti, Pierangelo, Wallace, Geoff, Weisenberg, Judy, Wilfong, Angus, Wirrell, Elaine C., Zafar, Muhammad, Zolnowska, Marta, Knight, Elia M Pestana, Amin, Sam, Bahi-Buisson, Nadia, Benke, Tim A, Cross, J Helen, Demarest, Scott T, Olson, Heather E, Specchio, Nicola, Fleming, Thomas R, Aimetti, Alex A, Gasior, Maciej, and Devinsky, Orrin

Published
2022
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9. Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group

Author

Kossoff, Eric H, Zupec‐Kania, Beth A, Auvin, Stéphane, Ballaban‐Gil, Karen R, Bergqvist, AG Christina, Blackford, Robyn, Buchhalter, Jeffrey R, Caraballo, Roberto H, Cross, J Helen, Dahlin, Maria G, Donner, Elizabeth J, Guzel, Orkide, Jehle, Rana S, Klepper, Joerg, Kang, Hoon‐Chul, Lambrechts, Danielle A, Liu, YM Christiana, Nathan, Janak K, Nordli, Douglas R, Pfeifer, Heidi H, Rho, Jong M, Scheffer, Ingrid E, Sharma, Suvasini, Stafstrom, Carl E, Thiele, Elizabeth A, Turner, Zahava, Vaccarezza, Maria M, Louw, Elles JTM, Veggiotti, Pierangelo, Wheless, James W, Wirrell, Elaine C, Foundation, The Charlie, Friends, Matthew's, and Society, the Practice Committee of the Child Neurology

Subjects
Biomedical and Clinical Sciences, Oncology and Carcinogenesis, Clinical Trials and Supportive Activities, Clinical Research, Complementary and Integrative Health, Nutrition, Charlie Foundation, Matthew's Friends, Practice Committee of the Child Neurology Society, Children, Diet, Epilepsy, Guideline, Ketogenic, Clinical sciences, Neurosciences, Biological psychology
Abstract

Ketogenic dietary therapies (KDTs) are established, effective nonpharmacologic treatments for intractable childhood epilepsy. For many years KDTs were implemented differently throughout the world due to lack of consistent protocols. In 2009, an expert consensus guideline for the management of children on KDT was published, focusing on topics of patient selection, pre-KDT counseling and evaluation, diet choice and attributes, implementation, supplementation, follow-up, side events, and KDT discontinuation. It has been helpful in outlining a state-of-the-art protocol, standardizing KDT for multicenter clinical trials, and identifying areas of controversy and uncertainty for future research. Now one decade later, the organizers and authors of this guideline present a revised version with additional authors, in order to include recent research, especially regarding other dietary treatments, clarifying indications for use, side effects during initiation and ongoing use, value of supplements, and methods of KDT discontinuation. In addition, authors completed a survey of their institution's practices, which was compared to responses from the original consensus survey, to show trends in management over the last 10 years.

Published
2018

16. How have the recent updated epilepsy classifications impacted on diagnosis and treatment?

Author

Wirrell, Elaine C, Riney, Kate, Specchio, Nicola, and Zuberi, Sameer M

Abstract

Epilepsies are a diverse group of disorders which differ regarding prognosis for seizure control and associated comorbidities. Accurate classification is critical to choose the highest yield investigations and best therapeutic options and to provide the most accurate prognoses regarding the expected degree of seizure control, possible remission, and risk of associated comorbidities to patients and their families. This article reviews the recent updates in epilepsy classification to illustrate how accurate classification impacts care for persons with epilepsy. The authors discuss the ILAE 2017 Classification of the Epilepsies along with the modification of the classification for neonatal seizures and epilepsies. They also discuss the ILAE position papers on Epilepsy syndromes in neonates and infants and children of variable age and the Idiopathic Generalized Epilepsies. Accurate epilepsy classification allows selection of the highest yield investigations, choice of optimal therapies, and accurate prognostication of seizures (likelihood of response to antiseizure treatments and likelihood of remission with age), as well as comorbidities (likelihood, type, and severity). As we move into the era of disease modifying therapy, early accurate identification of underlying causes with timely introduction of specific treatments will be crucial to lessen the severity of epilepsy, with improved seizure control and attenuation of associated comorbidities. [ABSTRACT FROM AUTHOR]

Published
2023
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17. Clinical presentation and evaluation of epilepsy with eyelid myoclonia: Results of an international expert consensus panel

Author

Smith, Kelsey M., primary, Wirrell, Elaine C., additional, Andrade, Danielle M., additional, Choi, Hyunmi, additional, Trenité, Dorothée Kasteleijn‐Nolst, additional, Jones, Hannah, additional, Knupp, Kelly G., additional, Mugar, Jon, additional, Nordli, Douglas R., additional, Riva, Antonella, additional, Stern, John M., additional, Striano, Pasquale, additional, Thiele, Elizabeth A., additional, and Zawar, Ifrah, additional

Published
2023
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18. Management of epilepsy with eyelid myoclonia: Results of an international expert consensus panel

Author

Smith, Kelsey M., primary, Wirrell, Elaine C., additional, Andrade, Danielle M., additional, Choi, Hyunmi, additional, Trenité, Dorothée Kasteleijn‐Nolst, additional, Jones, Hannah, additional, Knupp, Kelly G., additional, Mugar, Jon, additional, Nordli, Douglas R., additional, Riva, Antonella, additional, Stern, John M., additional, Striano, Pasquale, additional, Thiele, Elizabeth A., additional, and Zawar, Ifrah, additional

Published
2023
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21. A Comprehensive Narrative Review of Epilepsy with Eyelid Myoclonia

Author

Smith, Kelsey M., primary, Wirrell, Elaine C., additional, Andrade, Danielle M., additional, Choi, Hyunmi, additional, Trenité, Dorothée Kasteleijn-Nolst, additional, Knupp, Kelly G., additional, Nordli, Douglas R., additional, Riva, Antonella, additional, Stern, John M., additional, Striano, Pasquale, additional, Thiele, Elizabeth A., additional, and Zawar, Ifrah, additional

Published
2023
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28. Considerations for determining the efficacy of new antiseizure medications in children age 1 month to younger than 2 years

Author

French, Jacqueline A., primary, Cleary, Elena, additional, Dlugos, Dennis, additional, Farfel, Gail, additional, Farrell, Kathleen, additional, Gidal, Barry, additional, Grzeskowiak, Caitlin L., additional, Gurrell, Rachel, additional, Harden, Cynthia, additional, Stalvey, Tracy J., additional, Tsai, Julia, additional, Wirrell, Elaine C., additional, Blum, David, additional, and Fountain, Nathan, additional

Published
2022
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32. Value of genetic testing for pediatric epilepsy: Driving earlier diagnosis of ceroid lipofuscinosis type 2 Batten disease

Author

Leal‐Pardinas, Fernanda, primary, Truty, Rebecca, additional, McKnight, Dianalee A., additional, Johnson, Britt, additional, Morales, Ana, additional, Bristow, Sara L., additional, Yar Pang, Tiffany, additional, Cohen‐Pfeffer, Jessica, additional, Izzo, Emanuela, additional, Sankar, Raman, additional, Koh, Sookyong, additional, Wirrell, Elaine C., additional, Millichap, John J., additional, and Aradhya, Swaroop, additional

Published
2022
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33. ILAE definition of the Idiopathic Generalized Epilepsy Syndromes: Position statement by the ILAE Task Force on Nosology and Definitions

Author

Hirsch, Edouard, primary, French, Jacqueline, additional, Scheffer, Ingrid E., additional, Bogacz, Alicia, additional, Alsaadi, Taoufik, additional, Sperling, Michael R., additional, Abdulla, Fatema, additional, Zuberi, Sameer M., additional, Trinka, Eugen, additional, Specchio, Nicola, additional, Somerville, Ernest, additional, Samia, Pauline, additional, Riney, Kate, additional, Nabbout, Rima, additional, Jain, Satish, additional, Wilmshurst, Jo M., additional, Auvin, Stephane, additional, Wiebe, Samuel, additional, Perucca, Emilio, additional, Moshé, Solomon L., additional, Tinuper, Paolo, additional, Wirrell, Elaine C., additional, Adikaibe, Drs Birinus, additional, Al Baradi, Raidah, additional, Andrade, Danielle, additional, Bast, Thomas, additional, Beydoun, Ahmed, additional, Bien, Christian, additional, Caraballo, Roberto, additional, Carolina Coan, Ana, additional, Connolly, Mary, additional, Dunne, John, additional, Haut, Sheryl, additional, Jansen, Floor, additional, Jobst, Barbara, additional, Kalviainen, Reetta, additional, Kakooza, Angela, additional, Kato, Mitsuhiro, additional, Knupp, Kelly, additional, Kochen, Silvia, additional, Lagae, Lieven, additional, Carlos Mayor, Luis, additional, Okujava, Natela, additional, Radakishnan, Kurupath, additional, Roulet‐Perez, Eliane, additional, Rios, Loreto, additional, Sadleir, Lynette, additional, Juan‐Orta, Daniel San, additional, Serratosa, Jose, additional, Shellhaas, Renee, additional, Tsai, Meng‐Han, additional, Udani, Vrajesh, additional, Yue‐Hua Zhang,, Helen, additional, and Zhou., Dong, additional

Published
2022
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34. International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions

Author

Specchio, Nicola, primary, Wirrell, Elaine C., additional, Scheffer, Ingrid E., additional, Nabbout, Rima, additional, Riney, Kate, additional, Samia, Pauline, additional, Guerreiro, Marilisa, additional, Gwer, Sam, additional, Zuberi, Sameer M., additional, Wilmshurst, Jo M., additional, Yozawitz, Elissa, additional, Pressler, Ronit, additional, Hirsch, Edouard, additional, Wiebe, Samuel, additional, Cross, Helen J., additional, Perucca, Emilio, additional, Moshé, Solomon L., additional, Tinuper, Paolo, additional, and Auvin, Stéphane, additional

Published
2022
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36. International League Against Epilepsy classification and definition of epilepsy syndromes with onset at a variable age: position statement by the ILAE Task Force on Nosology and Definitions

Author

Riney, Kate, primary, Bogacz, Alicia, additional, Somerville, Ernest, additional, Hirsch, Edouard, additional, Nabbout, Rima, additional, Scheffer, Ingrid E., additional, Zuberi, Sameer M., additional, Alsaadi, Taoufik, additional, Jain, Satish, additional, French, Jacqueline, additional, Specchio, Nicola, additional, Trinka, Eugen, additional, Wiebe, Samuel, additional, Auvin, Stéphane, additional, Cabral‐Lim, Leonor, additional, Naidoo, Ansuya, additional, Perucca, Emilio, additional, Moshé, Solomon L., additional, Wirrell, Elaine C., additional, and Tinuper, Paolo, additional

Published
2022
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37. Methodology for classification and definition of epilepsy syndromes with list of syndromes: Report of the ILAE Task Force on Nosology and Definitions

Author

Wirrell, Elaine C., primary, Nabbout, Rima, additional, Scheffer, Ingrid E., additional, Alsaadi, Taoufik, additional, Bogacz, Alicia, additional, French, Jacqueline A., additional, Hirsch, Edouard, additional, Jain, Satish, additional, Kaneko, Sunao, additional, Riney, Kate, additional, Samia, Pauline, additional, Snead, O. Carter, additional, Somerville, Ernest, additional, Specchio, Nicola, additional, Trinka, Eugen, additional, Zuberi, Sameer M., additional, Balestrini, Simona, additional, Wiebe, Samuel, additional, Cross, J. Helen, additional, Perucca, Emilio, additional, Moshé, Solomon L., additional, and Tinuper, Paolo, additional

Published
2022
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38. Safety and efficacy of ganaxolone in patients with CDKL5 deficiency disorder: results from the double-blind phase of a randomised, placebo-controlled, phase 3 trial

Author

Knight, Elia M Pestana, primary, Amin, Sam, additional, Bahi-Buisson, Nadia, additional, Benke, Tim A, additional, Cross, J Helen, additional, Demarest, Scott T, additional, Olson, Heather E, additional, Specchio, Nicola, additional, Fleming, Thomas R, additional, Aimetti, Alex A, additional, Gasior, Maciej, additional, Devinsky, Orrin, additional, Belousova, Elena, additional, Belyaev, Oleg, additional, Ben-Zeev, Bruria, additional, Brunklaus, Andreas, additional, Ciliberto, Michael A., additional, Darra, Francesca, additional, Davis, Ronald, additional, De Giorgis, Valentina, additional, Doronina, Olga, additional, Fahey, Michael, additional, Guerrini, Renzo, additional, Heydemann, Peter, additional, Khaletskaya, Olga, additional, Lisewski, Pawel, additional, Marsh, Eric D., additional, Moosa, Ahsan N., additional, Perry, Scott, additional, Philip, Sunny, additional, Rajaraman, Rajsekar R., additional, Renfroe, Ben, additional, Saneto, Russell P., additional, Scheffer, Ingrid E., additional, Sogawa, Yoshimi, additional, Suter, Bernhardt, additional, Sweney, Matthew T., additional, Tarquinio, Daniel, additional, Veggiotti, Pierangelo, additional, Wallace, Geoff, additional, Weisenberg, Judy, additional, Wilfong, Angus, additional, Wirrell, Elaine C., additional, Zafar, Muhammad, additional, and Zolnowska, Marta, additional

Published
2022
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39. Sulthiame

Author

Claassen, Curtis, Wirrell, Elaine C., and Panayiotopoulos, C. P., editor

Published
2010
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42. Contributors

Author

Aaen, Gregory S., primary, Abend, Nicholas Scott, additional, Abou-Hamden, Amal, additional, Allen, Jeffrey C., additional, Amato, Anthony A., additional, Amlie-Lefond, Catherine, additional, Ashwal, Stephen, additional, Bailey, Russell C., additional, Bale, James F., additional, Banwell, Brenda, additional, Barañano, Kristin W., additional, Barkovich, A. James, additional, Barohn, Richard J., additional, Bartels, Ute K., additional, Bartnik-Olson, Brenda, additional, Barzilai, Ori, additional, Bassuk, Alexander, additional, Bearden, David R., additional, Ben-Sira, Liat, additional, Bernard, Timothy J., additional, Berry-Kravis, Elizabeth, additional, Beslow, Lauren A., additional, Biegel, Jaclyn A., additional, Billinghurst, Lori, additional, Birnbaum, Angela K., additional, Blackburn, Joanna S., additional, Bobowski, Nuala, additional, Boire, Adrienne, additional, Bönnemann, Carsten G., additional, Bonifacio, Sonia L., additional, Bonthius, Daniel J., additional, Borcherding, Breck, additional, Branchford, Brian R., additional, Brandsema, John, additional, Brennan, Kathryn M., additional, Brenton, J. Nicholas, additional, Brooks-Kayal, Amy R., additional, Brown, Lawrence W., additional, Buchalter, Jeffrey, additional, Camfield, Carol S., additional, Camfield, Peter R., additional, Campoy, Cristina, additional, Carpenter, Jessica L., additional, Chang, Taeun, additional, Chau, Vann, additional, Chi, Susan N., additional, Chiriboga, Claudia A., additional, Cho, Yoon-Jae, additional, Christian, Cindy W., additional, Chrestian, Nicholas, additional, Cilio, Maria Roberta, additional, Clark, Robin D., additional, Cohen, Bruce H., additional, Cohn, Ronald D., additional, Connolly, Anne M., additional, Constable, Todd, additional, Constantini, Shlomi, additional, Conway, Jeannine M., additional, Coulter, David L., additional, Cowan, Tina M., additional, Dale, Russell C., additional, Darbro, Benjamin, additional, Darras, Basil T., additional, Dastgir, Jahannaz, additional, De Meirleir, Linda, additional, De Vivo, Darryl C., additional, de Vries, Linda S., additional, Deisch, Jeremy K., additional, Deltenre, Paul, additional, Desai, Jay, additional, Descartes, Maria, additional, deVeber, Gabrielle, additional, Dhamne, Sameer C., additional, Diaz, Jullianne, additional, DiMauro, Salvatore, additional, Dobyns, William B., additional, Doherty, Dan, additional, Donner, Elizabeth J., additional, Dosenbach, Nico U.F., additional, Dowling, James J., additional, Drake, James M., additional, Ejerskov, Cecile, additional, Engel, Andrew G., additional, Enns, Gregory M., additional, Escolano-Margarit, María Victoria, additional, Etzion, Iris, additional, Fatemi, S. Ali, additional, Fehlings, Darcy L., additional, Feinberg, Michelle Lauren, additional, Ferriero, Donna M., additional, Filipek, Pauline A., additional, Finkel, Richard S., additional, Fisher, Paul G., additional, Flanigan, Kevin, additional, Foreman, Nicholas K., additional, Franco, Israel, additional, Frank, Yitzchak, additional, Fredrick, Douglas R., additional, Freeze, Hudson H., additional, Fuente-Mora, Cristina, additional, Furman, Joseph M., additional, Gallagher, Renata C., additional, Garel, Catherine, additional, Gertsch, Emily, additional, Gilbert, Donald L., additional, Gilles, Elizabeth E., additional, Giza, Christopher C., additional, Glaser, Carol A., additional, Glass, Hannah C., additional, Glauser, Tracy, additional, Glykys, Joseph, additional, Goldstein, Amy, additional, Gonorazky, Hernan Dario, additional, Gonzalez, Rodolfo, additional, Goodkin, Howard P., additional, Graham, John M., additional, Greninger, Alexander L., additional, Gronseth, Gary, additional, Gropman, Andrea L., additional, Grundy, Richard, additional, Guerrini, Renzo, additional, Gupta, Nalin, additional, Hahn, Jin S., additional, Hamblin, Milton H., additional, Hani, Abeer J., additional, Hanmantgad, Sharyu, additional, Harbert, Mary J., additional, Harini, Chellamani, additional, Harriott, Andrea M., additional, Heatwole, Chad, additional, Hershey, Andrew D., additional, Hirtz, Deborah G., additional, Holmes, Gregory L., additional, Holshouser, Barbara A., additional, Hurwitz, Kathleen A., additional, Hwang, Eugene, additional, Ichord, Rebecca N., additional, Jafar-Nejad, Paymaan, additional, Jain, Sejal V., additional, Jordan, Lori, additional, Kabbouche, Marielle A., additional, Kacperski, Joanne, additional, Kang, Peter B., additional, Kariannis, Matthias A., additional, Kaufmann, Horacio, additional, Kaye, Harper L., additional, Keating, Robert, additional, Kennedy, Colin R., additional, Khakoo, Yasmin, additional, Kirton, Adam, additional, Kissel, John T., additional, Knupp, Kelly G., additional, Korf, Bruce R., additional, Kossoff, Eric H., additional, Kothare, Sanjeev V., additional, Kupfer, Oren, additional, LaFrance, W. Curt, additional, Latal, Beatrice, additional, Leber, Steven M., additional, Lee, Jean-Pyo, additional, Leppik, Ilo E., additional, Lerman-Sagie, Tally, additional, Lerner, Jason T., additional, Leventer, Richard J., additional, Licht, Daniel J., additional, Lichter-Konecki, Uta, additional, Lidar, Zvi, additional, Liem, Djin Gie, additional, Loddenkemper, Tobias, additional, Long, Roger K., additional, Luc, Quyen N., additional, Mackay, Mark, additional, Majnemer, Annette, additional, Makhani, Naila, additional, Malinger, Gustavo, additional, Mandelbaum, David E., additional, Maricich, Stephen M., additional, Maski, Kiran P., additional, Mathur, Mudit, additional, Matthews, Dennis J., additional, McMahon, Kelly, additional, DeMara-Hoth, Megan B., additional, Mendelsohn, Bryce, additional, Mennella, Julie A., additional, Ment, Laura R., additional, Mercuri, Eugenio, additional, Michelson, David J., additional, Mikati, Mohamad A., additional, Mikhail, Fady M., additional, Miller, Steven Paul, additional, Milunsky, Jeff M., additional, Mink, Jonathan W., additional, Mirzaa, Ghayda M., additional, Mitchell, Wendy G., additional, Mohan, Michael A., additional, Mohassel, Payam, additional, Moharir, Mahendranath, additional, Monani, Umrao R., additional, Monje Deisseroth, Michelle, additional, Moodley, Manikum, additional, Mower, Andrew, additional, Moxley, Richard T., additional, Mueller, Sabine, additional, Muotri, Alysson R., additional, Nagamani, Sandesh C.S., additional, Narayanan, Mohan J., additional, Narayanan, Vinodh, additional, Nass, Ruth D., additional, Neul, Jeffrey L., additional, Nevo, Yoram, additional, Ng, Bobby G., additional, Nickels, Katherine C., additional, Nimmo, Graeme A.M., additional, Noetzel, Michael J., additional, Norcliffe-Kaufmann, Lucy, additional, Nordli, Douglas R., additional, Nowak-Göttl, Ulrike, additional, O'Brien, Hope L., additional, Oleszek, Joyce, additional, Oskoui, Maryam, additional, Paciorkowski, Alex R., additional, Packer, Roger J., additional, Packman, Seymour, additional, Palma, Jose-Alberto, additional, Pardo, Andrea C., additional, Parsons, Julie A., additional, Partridge, John Colin, additional, Pastores, Gregory M., additional, Patterson, Marc C., additional, Pearce, William J., additional, Pearl, Phillip L., additional, Penner, Melanie, additional, Percival, Leila, additional, Pereira, Marcia, additional, Pfister, Stefan M., additional, Phillips, John, additional, Plecko, Barbara, additional, Plioplys, Sigita, additional, Poduri, Annapurna, additional, Poisson, Sharon, additional, Pomeroy, Scott L., additional, Poretti, Andrea, additional, Powers, Scott W., additional, Pranzatelli, Michael R., additional, Przekop, Allison, additional, Rabie, Malcolm, additional, Rangasamy, Sampathkumar, additional, Raymond, Gerald V., additional, Reddy, Alyssa T., additional, Rendleman, Rebecca L., additional, Rho, Jong M., additional, Rodan, Lance H., additional, Roddy, Sarah M., additional, Rogers, Elizabeth E., additional, Rosenthal, Stephen M., additional, Rosman, N. Paul, additional, Ross, M. Elizabeth, additional, Rotenberg, Alexander, additional, Rust, Robert S., additional, Sanchez, Cheryl P., additional, Sanchez, Pedro, additional, Sánchez Fernández, Iván, additional, Sands, Tristan T., additional, Sanger, Terence D., additional, Sannagowdara, Kumar, additional, Scheinost, Dustin, additional, Scher, Mark S., additional, Schor, Nina F., additional, Schrauwen, Isabelle, additional, Segal, Michael M., additional, Seinfeld, Syndi, additional, Selcen, Duygu, additional, Seltzer, Laurie E., additional, Semrud-Clikeman, Margaret, additional, Shaw, Dennis W., additional, Shaywitz, Bennett A., additional, Shaywitz, Sally E., additional, Shellhaas, Renée A., additional, Sherr, Elliott H., additional, Sheth, Rita D., additional, Shevell, Michael I., additional, Shinnar, Shlomo, additional, Shofty, Ben, additional, Shu, Stanford K., additional, Shy, Michael E., additional, Silveira Moriyama, Laura, additional, Silvestri, Nicholas J., additional, Sims, Katherine B., additional, Singer, Harvey S., additional, Singhal, Nilika Shah, additional, Smith, Craig M., additional, Smith, Edward, additional, Smith, Stephen A., additional, Snyder, Evan Y., additional, Soul, Janet, additional, Spalink, Christy L., additional, Spencer, Karen A., additional, Stafstrom, Carl E., additional, Steinfeld, Robert, additional, Strober, Jonathan B., additional, Sullivan, Joseph, additional, Swaiman, Kenneth F., additional, Swoboda, Kathryn J., additional, Tate, Elizabeth D., additional, Tatum, William O., additional, Tein, Ingrid, additional, Tekulve, Kristyn, additional, Tenney, Jeffrey R., additional, Thiele, Elizabeth A., additional, Thompson-Stone, Robert, additional, Tochen, Laura, additional, Tormoehlen, Laura M., additional, Tran, Lily, additional, Trauner, Doris A., additional, Turnacioglu, Sinan O., additional, Ullrich, Nicole J., additional, Urion, David K., additional, Van Camp, Guy, additional, Van Hirtum-Das, Michèle, additional, van Karnebeek, Clara D.M., additional, Van Maldergem, Lionel, additional, Vanderver, Adeline, additional, Vitanza, Nicholas A., additional, von Rhein, Michael, additional, von Scheven, Emily, additional, Wagner, Ann, additional, Wainwright, Mark S., additional, Walker, Melissa A., additional, Walkup, John T., additional, Walsh, Laurence, additional, Walters-Sen, Lauren C., additional, Wang, Raymond Y., additional, Warner, Thomas T., additional, Whelan, Harry T., additional, Weinberg, Geoffrey A., additional, Wells, Elizabeth M., additional, Wheless, James W., additional, Wirrell, Elaine C., additional, Wisoff, Jeffrey H., additional, Wolf, Nicole I., additional, Wolfe, Gil I., additional, Wright, F. Virginia, additional, Wycliffe, Nathaniel D., additional, Yang, Michele L., additional, Yuskaitis, Christopher J., additional, Zoghbi, Huda Y., additional, and Zupanc, Mary L., additional

Published
2017
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43. Stiripentol for the treatment of seizures associated with Dravet syndrome in patients 6 months and older and taking clobazam.

Author

Vasquez, Alejandra, Wirrell, Elaine C, and Youssef, Paul E

Abstract

Stiripentol (STP) is a structurally unique molecule with anticonvulsant and neuroprotective properties in animal and human studies. STP enhances gamma-aminobutyric acid (GABA)ergic neurotransmission and inhibits multiple hepatic isoenzymes (i.e. cytochrome P450 system) involved in the metabolism of other antiseizure medications (ASMs) potentiating their anticonvulsant effects and has proven to be a promising therapy in Dravet Syndrome (DS). The authors review randomized clinical trials and observational studies showing STP efficacy, safety, and tolerability when used as adjunctive therapy with VPA and clobazam in patients with DS. Moreover, they include recent evidence of its use in patients<2 years of age. Evidence on STP demonstrates clinically meaningful efficacy in both short and long term in patients with DS. In addition to reducing convulsive seizure frequency, STP also markedly reduces the number of status epilepticus episodes and associated medical complications which are more common in younger children. STP adverse effects are generally not severe and often resolve following STP dose reduction or adjustments of concomitant ASMs. STP is approved by the FDA for children aged 6 months and older with DS who are also taking clobazam, making it the only DS-specific ASM for children under age 1 year. [ABSTRACT FROM AUTHOR]

Published
2023
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46. Dravet Syndrome as an Example of Precision Medicine in Epilepsy.

Author

Sullivan, Joseph and Wirrell, Elaine C.

Subjects
*INDIVIDUALIZED medicine, *SYNDROMES, *LENNOX-Gastaut syndrome, *EPILEPSY, *BRAIN diseases, *EARLY diagnosis, *PEOPLE with epilepsy
Abstract

Dravet syndrome (DS) is a drug-resistant, early-onset, developmental and epileptic encephalopathy where there have been many recently approved therapies with many more in development. With the availability of more syndrome specific treatment options coupled with an earlier diagnosis, DS is well-positioned to be an example of how a precise syndromic diagnosis can guide treatment choices and improve overall outcomes and also allow for the development of potential disease modifying therapies to address more than just seizures. In this review we summarize the current state of DS approved therapies and those that are in various stages of development. [ABSTRACT FROM AUTHOR]

Published
2023
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