Leslie E Grayson1, Jurriaan M Peters2, Tarrant McPherson3, Darcy A Krueger4, Mustafa Sahin5, Joyce Y Wu6, Hope A Northrup7, Brenda Porter8, Gary R Cutter3, Sarah E O'Kelley9, Jessica Krefting1, Scellig S Stone10, Joseph R Madsen10, Aria Fallah11, Jeffrey P Blount12, Howard L Weiner13, E Martina Bebin14. 1. Department of Neurology, University of Alabama at Birmingham, Birmingham, Alabama. 2. Localization Laboratory, Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts. 3. Department of Biostatistics, University of Alabama at Birmingham, Birmingham, Alabama. 4. Department of Neurology, Cincinnati Children's Hospital Medical Center, and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio. 5. Department of Neurology and the F.M. Kirby Neurobiology Center, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts. 6. Division of Pediatric Neurology, UCLA Mattel Children's Hospital, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California. 7. Department of Pediatrics, McGovern Medical School, University of Texas Health Science Center at Houston, Houston, Texas. 8. Department of Neurology, The Stanford University Medical Center, Stanford, California. 9. Department of Psychology, University of Alabama at Birmingham, Birmingham, Alabama. 10. Division of Pediatric Neurosurgery, Department of Neurosurgery, Boston Children's Hospital and Harvard Medical School, Boston, Massachusetts. 11. Department of Neurosurgery, Division of Pediatric Neurosurgery, University of California Los Angeles Mattel Children's Hospital, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, California. 12. Department of Neurosurgery, Division of Pediatric Neurosurgery, Children's Hospital of Alabama and University of Alabama at Birmingham, Birmingham, Alabama. 13. Division of Pediatric Neurosurgery, Department of Surgery, Texas Children's Hospital and Department of Neurosurgery, Baylor College of Medicine, Houston, Texas. 14. Department of Neurology, University of Alabama at Birmingham, Birmingham, Alabama. Electronic address: ebebin@uab.edu.
Abstract
BACKGROUND: To determine if early epilepsy surgery mitigates detrimental effects of refractory epilepsy on development, we investigated surgical and neurodevelopmental outcomes in children with tuberous sclerosis complex who underwent surgery before age two years. METHODS: Prospective multicenter observational study of 160 children with tuberous sclerosis complex. Surgical outcome was determined for the seizure type targeted by surgery. We obtained Vineland Adaptive Behavior Scales, Second Edition (Vineland-II); Mullen Scales of Early Learning; and Preschool Language Scales, Fifth Edition, at age three, six, nine, 12, 18, 24, and 36 months. Surgical cases were compared with children without seizures, with controlled seizures, and with medically refractory seizures. RESULTS: Nineteen children underwent surgery (median age 17 months, range 3.7 to 21.3), and mean follow-up was 22.8 months (range 12 to 48). Surgical outcomes were favorable in 12 (63%, Engel I-II) and poor in seven (37%, Engel III-IV). Nine (47%) had new or ongoing seizures distinct from those surgically targeted. All children with seizures demonstrated longitudinal decline or attenuated gains in neurodevelopment, the surgical group scoring the lowest. Favorable surgical outcome was associated with increased Mullen Scales of Early Learning receptive and expressive language subscores compared with the medically refractory seizure group. A nonsignificant but consistent pattern of improvement with surgery was seen in all tested domains. CONCLUSIONS: These pilot data show neurodevelopmental gains in some domains following epilepsy surgery. A properly powered, prospective multicenter observational study of early epilepsy surgery is needed, using both surgical and developmental outcome metrics.
BACKGROUND: To determine if early epilepsy surgery mitigates detrimental effects of refractory epilepsy on development, we investigated surgical and neurodevelopmental outcomes in children with tuberous sclerosis complex who underwent surgery before age two years. METHODS: Prospective multicenter observational study of 160 children with tuberous sclerosis complex. Surgical outcome was determined for the seizure type targeted by surgery. We obtained Vineland Adaptive Behavior Scales, Second Edition (Vineland-II); Mullen Scales of Early Learning; and Preschool Language Scales, Fifth Edition, at age three, six, nine, 12, 18, 24, and 36 months. Surgical cases were compared with children without seizures, with controlled seizures, and with medically refractory seizures. RESULTS: Nineteen children underwent surgery (median age 17 months, range 3.7 to 21.3), and mean follow-up was 22.8 months (range 12 to 48). Surgical outcomes were favorable in 12 (63%, Engel I-II) and poor in seven (37%, Engel III-IV). Nine (47%) had new or ongoing seizures distinct from those surgically targeted. All children with seizures demonstrated longitudinal decline or attenuated gains in neurodevelopment, the surgical group scoring the lowest. Favorable surgical outcome was associated with increased Mullen Scales of Early Learning receptive and expressive language subscores compared with the medically refractory seizure group. A nonsignificant but consistent pattern of improvement with surgery was seen in all tested domains. CONCLUSIONS: These pilot data show neurodevelopmental gains in some domains following epilepsy surgery. A properly powered, prospective multicenter observational study of early epilepsy surgery is needed, using both surgical and developmental outcome metrics.
Authors: Aria Fallah; Shaun D Rodgers; Alexander G Weil; Sumeet Vadera; Alireza Mansouri; Mary B Connolly; Philippe Major; Tracy Ma; Orrin Devinsky; Howard L Weiner; Jorge A Gonzalez-Martinez; William E Bingaman; Imad Najm; Ajay Gupta; John Ragheb; Sanjiv Bhatia; Paul Steinbok; Christopher D Witiw; Elysa Widjaja; O Carter Snead; James T Rutka Journal: Neurosurgery Date: 2015-10 Impact factor: 4.654
Authors: Jamie K Capal; Beatriz Bernardino-Cuesta; Paul S Horn; Donna Murray; Anna Weber Byars; Nicole M Bing; Bridget Kent; Deborah A Pearson; Mustafa Sahin; Darcy A Krueger Journal: Epilepsy Behav Date: 2017-04-28 Impact factor: 2.937
Authors: J Y Wu; N Salamon; H E Kirsch; M M Mantle; S S Nagarajan; L Kurelowech; M H Aung; R Sankar; W D Shields; G W Mathern Journal: Neurology Date: 2010-02-02 Impact factor: 9.910
Authors: F E Jansen; K L Vincken; A Algra; P Anbeek; O Braams; M Nellist; B A Zonnenberg; A Jennekens-Schinkel; A van den Ouweland; D Halley; A C van Huffelen; O van Nieuwenhuizen Journal: Neurology Date: 2007-11-21 Impact factor: 9.910
Authors: Catherine J Chu-Shore; Philippe Major; Susana Camposano; David Muzykewicz; Elizabeth A Thiele Journal: Epilepsia Date: 2009-12-22 Impact factor: 5.864
Authors: Peter E Davis; Rajna Filip-Dhima; Georgios Sideridis; Jurriaan M Peters; Kit Sing Au; Hope Northrup; E Martina Bebin; Joyce Y Wu; Darcy Krueger; Mustafa Sahin Journal: Pediatrics Date: 2017-11-03 Impact factor: 7.124
Authors: Robert J Bollo; Stephen P Kalhorn; Chad Carlson; Veronique Haegeli; Orrin Devinsky; Howard L Weiner Journal: Neurosurg Focus Date: 2008-09 Impact factor: 4.047
Authors: Fiona M Baumer; Jurriaan M Peters; Sean Clancy; Anna K Prohl; Sanjay P Prabhu; Benoit Scherrer; Floor E Jansen; Kees P J Braun; Mustafa Sahin; Aymeric Stamm; Simon K Warfield Journal: Cereb Cortex Date: 2018-10-01 Impact factor: 5.357
Authors: S Katie Z Ihnen; Jamie K Capal; Paul S Horn; Molly Griffith; Mustafa Sahin; E Martina Bebin; Joyce Y Wu; Hope Northrup; Darcy A Krueger Journal: Pediatr Neurol Date: 2021-07-06 Impact factor: 4.210