John R Mytinger1, Dara V F Albert2, Jaime D Twanow2, Jorge Vidaurre2, Yubo Tan3, Guy N Brock4, Adam P Ostendorf2. 1. Division of Pediatric Neurology, Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio. Electronic address: john.mytinger@nationwidechildrens.org. 2. Division of Pediatric Neurology, Department of Pediatrics, Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio. 3. Department of Biomedical Informatics, College of Medicine, The Ohio State University, Columbus, Ohio; Center for Biostatistics, Wexner Medical Center, The Ohio State University, Columbus, Ohio; Biostatistics Resource at NCH (BRANCH), Nationwide Children's Hospital and The Ohio State University, Columbus, Ohio. 4. Department of Biomedical Informatics, College of Medicine, The Ohio State University, Columbus, Ohio; Center for Biostatistics, Wexner Medical Center, The Ohio State University, Columbus, Ohio; Biostatistics Resource at NCH (BRANCH), Nationwide Children's Hospital and The Ohio State University, Columbus, Ohio; Department of Pediatrics, Nationwide Children's Hospital and The Ohio State University, Columbus, Ohio.
Abstract
BACKGROUND: We implemented an infantile spasms management guideline recommending standard therapies and, early start of next treatment. After six years, we determined (1) our compliance with standard therapies, (2) time to next treatment, and (3) rate of initial and three-month electroclinical remission with first, second, and third treatments. METHODS: This is a retrospective record review of newly diagnosed spasms from September 2012 to September 2018, with the onset age of two months to two years. RESULTS: Standard therapies (hormone or vigabatrin) were the first treatments in 114 of 115 consecutive patients. The second and third treatments were started within 14 days of failed treatment in only 21% and 24%, respectively. Remission with the first and second treatments was similar (41% and 40%). Remission was lower for the third treatment (15%), although higher if standard therapy was used (36%). Initial and three-month remission by the first treatment was significantly higher for adrenocorticotropic hormone (ACTH, 66% and 79%, respectively) and prednisolone (53% and 83%, respectively) than for vigabatrin (19% and 40%, respectively). There were no significant differences in patient characteristics or rates of remission between ACTH and prednisolone. CONCLUSIONS: Although we achieved excellent compliance with standard therapies as initial treatment, a next treatment often started after two weeks. Given the superiority of hormone therapies over vigabatrin and standard therapies over nonstandard therapies, as well as the potentially negative impact of delays in effective treatment, future interventions need to focus on increasing the use of hormone over vigabatrin (for patients without tuberous sclerosis complex), use of standard therapies as second and third treatments, and reducing delays to next treatment.
BACKGROUND: We implemented an infantile spasms management guideline recommending standard therapies and, early start of next treatment. After six years, we determined (1) our compliance with standard therapies, (2) time to next treatment, and (3) rate of initial and three-month electroclinical remission with first, second, and third treatments. METHODS: This is a retrospective record review of newly diagnosed spasms from September 2012 to September 2018, with the onset age of two months to two years. RESULTS: Standard therapies (hormone or vigabatrin) were the first treatments in 114 of 115 consecutive patients. The second and third treatments were started within 14 days of failed treatment in only 21% and 24%, respectively. Remission with the first and second treatments was similar (41% and 40%). Remission was lower for the third treatment (15%), although higher if standard therapy was used (36%). Initial and three-month remission by the first treatment was significantly higher for adrenocorticotropic hormone (ACTH, 66% and 79%, respectively) and prednisolone (53% and 83%, respectively) than for vigabatrin (19% and 40%, respectively). There were no significant differences in patient characteristics or rates of remission between ACTH and prednisolone. CONCLUSIONS: Although we achieved excellent compliance with standard therapies as initial treatment, a next treatment often started after two weeks. Given the superiority of hormone therapies over vigabatrin and standard therapies over nonstandard therapies, as well as the potentially negative impact of delays in effective treatment, future interventions need to focus on increasing the use of hormone over vigabatrin (for patients without tuberous sclerosis complex), use of standard therapies as second and third treatments, and reducing delays to next treatment.
Authors: Andrew L Lux; Stuart W Edwards; Eleanor Hancock; Anthony L Johnson; Colin R Kennedy; Richard W Newton; Finbar J K O'Callaghan; Christopher M Verity; John P Osborne Journal: Lancet Date: 2004 Nov 13-19 Impact factor: 79.321
Authors: Finbar J K O'Callaghan; Stuart W Edwards; Fabienne Dietrich Alber; Mario Cortina Borja; Eleanor Hancock; Anthony L Johnson; Colin R Kennedy; Marcus Likeman; Andrew L Lux; Mark T Mackay; Andrew A Mallick; Richard W Newton; Melinda Nolan; Ronit Pressler; Dietz Rating; Bernhard Schmitt; Christopher M Verity; John P Osborne Journal: Lancet Child Adolesc Health Date: 2018-08-29
Authors: Robert S Fisher; J Helen Cross; Carol D'Souza; Jacqueline A French; Sheryl R Haut; Norimichi Higurashi; Edouard Hirsch; Floor E Jansen; Lieven Lagae; Solomon L Moshé; Jukka Peltola; Eliane Roulet Perez; Ingrid E Scheffer; Andreas Schulze-Bonhage; Ernest Somerville; Michael Sperling; Elza Márcia Yacubian; Sameer M Zuberi Journal: Epilepsia Date: 2017-03-08 Impact factor: 5.864
Authors: Anup D Patel; Anne T Berg; Lori Billinghurst; Daniel Fain; Erin Fecske; Tim Feyma; Zachary Grinspan; Amy Houtrow; Sanjeev Kothare; Gogi Kumar; Erin Lee; Migvis Monduy; Diego Morita; Christina L Szperka; M Cristina Victorio; Ann Yeh; Jeffrey R Buchhalter Journal: Neurology Date: 2017-12-15 Impact factor: 9.910
Authors: John R Mytinger; Shaun A Hussain; Monica P Islam; John J Millichap; Anup D Patel; Nicole R Ryan; Jaime-Dawn E Twanow; Geoffrey L Heyer Journal: Epilepsy Res Date: 2015-07-28 Impact factor: 3.045
Authors: Kelly G Knupp; Erin Leister; Jason Coryell; Katherine C Nickels; Nicole Ryan; Elizabeth Juarez-Colunga; William D Gaillard; John R Mytinger; Anne T Berg; John Millichap; Douglas R Nordli; Sucheta Joshi; Renée A Shellhaas; Tobias Loddenkemper; Dennis Dlugos; Elaine Wirrell; Joseph Sullivan; Adam L Hartman; Eric H Kossoff; Zachary M Grinspan; Lorie Hamikawa Journal: Epilepsia Date: 2016-09-12 Impact factor: 5.864
Authors: Fiona M Baumer; John R Mytinger; Kerri Neville; Christina Briscoe Abath; Camilo A Gutierrez; Adam L Numis; Chellamani Harini; Zihuai He; Shaun A Hussain; Anne T Berg; Catherine J Chu; William D Gaillard; Tobias Loddenkemper; Archana Pasupuleti; Debopam Samanta; Rani K Singh; Nilika S Singhal; Courtney J Wusthoff; Elaine C Wirrell; Elissa Yozawitz; Kelly G Knupp; Renée A Shellhaas; Zachary M Grinspan Journal: Ann Neurol Date: 2022-04-28 Impact factor: 11.274
Authors: Zachary M Grinspan; John R Mytinger; Fiona M Baumer; Michael A Ciliberto; Bruce H Cohen; Dennis J Dlugos; Chellamani Harini; Shaun A Hussain; Sucheta M Joshi; Cynthia G Keator; Kelly G Knupp; Patricia E McGoldrick; Katherine C Nickels; Jun T Park; Archana Pasupuleti; Anup D Patel; Asim M Shahid; Renee A Shellhaas; Daniel W Shrey; Rani K Singh; Steven M Wolf; Elissa G Yozawitz; Christopher J Yuskaitis; Jeff L Waugh; Phillip L Pearl Journal: J Child Neurol Date: 2020-06-23 Impact factor: 1.987
Authors: Blanca Romero Milà; Kavyakantha Remakanthakurup Sindhu; John R Mytinger; Daniel W Shrey; Beth A Lopour Journal: Front Neurol Date: 2022-07-28 Impact factor: 4.086