Suresh S Pujar1, Marina M Martinos2, Mario Cortina-Borja3, W K Kling Chong4, Michelle De Haan2, Christopher Gillberg5, Brian G Neville6, Rod C Scott7, Richard F Chin8. 1. Clinical Neurosciences, UCL Great Ormond Street Institute of Child Health, London, UK; Clinical Neurosciences, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK; Young Epilepsy, Lingfield, UK. Electronic address: s.pujar@ucl.ac.uk. 2. Cognitive Neuroscience and Neuropsychiatry Program, UCL Great Ormond Street Institute of Child Health, London, UK. 3. Population, Policy and Practice Program, UCL Great Ormond Street Institute of Child Health, London, UK. 4. Department of Radiology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK. 5. Clinical Neurosciences, UCL Great Ormond Street Institute of Child Health, London, UK; Gillberg Neuropsychiatry Centre, University of Gothenburg, Gothenburg, Sweden. 6. Clinical Neurosciences, UCL Great Ormond Street Institute of Child Health, London, UK; Young Epilepsy, Lingfield, UK. 7. Clinical Neurosciences, UCL Great Ormond Street Institute of Child Health, London, UK; Clinical Neurosciences, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK; Young Epilepsy, Lingfield, UK; Department of Neurological Sciences, University of Vermont College of Medicine, Burlington, VT, USA. 8. Clinical Neurosciences, UCL Great Ormond Street Institute of Child Health, London, UK; Muir Maxwell Epilepsy Centre, Department of Child Life and Health, The University of Edinburgh, Edinburgh, UK.
Abstract
BACKGROUND: The prognosis of convulsive status epilepticus (CSE), a common childhood medical neurological emergency, is not well characterised. We aimed to investigate the long-term outcomes in a cohort of participants who previously had CSE. METHODS: In this prospective study, we followed up a population-based childhood CSE cohort from north London, UK (the north London convulsive status epilepticus surveillance study cohort; NLSTEPSS). We collected data from structured clinical neurological assessment, neurocognitive assessment (Wechsler Abbreviated Scale of Intelligence), brain MRI, medical records, and structured interviews with participants and their parents to determine neurological outcomes, with adverse outcome defined as presence of one or more of epilepsy (active or in remission), motor disability, intellectual disability, or statement of special educational needs. We applied multiple imputation to address missing data and performed binary logistic regression analyses on complete-case and imputed datasets to investigate sociodemographic and CSE factors associated with adverse outcomes. FINDINGS: Of 203 survivors (90% of inception cohort), 134 (66%) were assessed at a median follow-up of 8·9 years (IQR 8·2-9·5). The cumulative incidence of epilepsy was 24·7% (95% CI 16·2-35·6), with most (89%) emerging within 18 months after CSE. The cumulative incidence of epilepsy was lower in patients with prolonged febrile seizures (14·3%, 6·3-29·4) and survivors of acute symptomatic CSE (13·3%, 3·7-37·9) than in those of remote symptomatic CSE (45·5%, 21·3-72·0) and unclassified CSE (50·0%, 25·4-74·6). One participant (2·9%, 0·5-14·5) in the prolonged febrile seizures group developed temporal lobe epilepsy with mesial temporal sclerosis. The absence of fever at CSE was the only predictor of incident epilepsy (odds ratio [OR] 7·5, 95% CI 2·25-25·1). Motor and intellectual disability was seen predominantly in participants who had idiopathic and cryptogenic CSE (seven [36·8%, 95% CI 19·1-59·0] and 16 [84·2%, 62·4-94·5] of 19, respectively) and remote symptomatic CSE (33 [62·3%, 48·8-74·1] and 40 [75·5%, 62·4-85·1] of 53), and most of these participants had pre-existing disabilities. Pre-existing epilepsy was the only predictor of intellectual disability (OR 8·0, 95% CI 1·1-59·6). 51·5% (95% CI 43·1-59·8) of those followed up had a statement of special educational needs. INTERPRETATION: Childhood CSE is associated with substantial long-term neurological morbidity, but primarily in those who have epilepsy, neurological abnormalities, or both before the episode of CSE. Survivors without neurological abnormalities before CSE have favourable outcomes. FUNDING: BUPA Foundation, The Academy of Medical Sciences, Wellcome Trust, National Institute for Health Research, and Young Epilepsy.
BACKGROUND: The prognosis of convulsive status epilepticus (CSE), a common childhood medical neurological emergency, is not well characterised. We aimed to investigate the long-term outcomes in a cohort of participants who previously had CSE. METHODS: In this prospective study, we followed up a population-based childhood CSE cohort from north London, UK (the north London convulsive status epilepticus surveillance study cohort; NLSTEPSS). We collected data from structured clinical neurological assessment, neurocognitive assessment (Wechsler Abbreviated Scale of Intelligence), brain MRI, medical records, and structured interviews with participants and their parents to determine neurological outcomes, with adverse outcome defined as presence of one or more of epilepsy (active or in remission), motor disability, intellectual disability, or statement of special educational needs. We applied multiple imputation to address missing data and performed binary logistic regression analyses on complete-case and imputed datasets to investigate sociodemographic and CSE factors associated with adverse outcomes. FINDINGS: Of 203 survivors (90% of inception cohort), 134 (66%) were assessed at a median follow-up of 8·9 years (IQR 8·2-9·5). The cumulative incidence of epilepsy was 24·7% (95% CI 16·2-35·6), with most (89%) emerging within 18 months after CSE. The cumulative incidence of epilepsy was lower in patients with prolonged febrile seizures (14·3%, 6·3-29·4) and survivors of acute symptomatic CSE (13·3%, 3·7-37·9) than in those of remote symptomatic CSE (45·5%, 21·3-72·0) and unclassified CSE (50·0%, 25·4-74·6). One participant (2·9%, 0·5-14·5) in the prolonged febrile seizures group developed temporal lobe epilepsy with mesial temporal sclerosis. The absence of fever at CSE was the only predictor of incident epilepsy (odds ratio [OR] 7·5, 95% CI 2·25-25·1). Motor and intellectual disability was seen predominantly in participants who had idiopathic and cryptogenic CSE (seven [36·8%, 95% CI 19·1-59·0] and 16 [84·2%, 62·4-94·5] of 19, respectively) and remote symptomatic CSE (33 [62·3%, 48·8-74·1] and 40 [75·5%, 62·4-85·1] of 53), and most of these participants had pre-existing disabilities. Pre-existing epilepsy was the only predictor of intellectual disability (OR 8·0, 95% CI 1·1-59·6). 51·5% (95% CI 43·1-59·8) of those followed up had a statement of special educational needs. INTERPRETATION: Childhood CSE is associated with substantial long-term neurological morbidity, but primarily in those who have epilepsy, neurological abnormalities, or both before the episode of CSE. Survivors without neurological abnormalities before CSE have favourable outcomes. FUNDING: BUPA Foundation, The Academy of Medical Sciences, Wellcome Trust, National Institute for Health Research, and Young Epilepsy.
Authors: Marina M Martinos; Suresh Pujar; Helen O'Reilly; Michelle de Haan; Brian G R Neville; Rod C Scott; Richard F M Chin Journal: Epilepsy Behav Date: 2019-04-19 Impact factor: 2.937
Authors: Iván Sánchez Fernández; Nicholas S Abend; Marta Amengual-Gual; Anne Anderson; Ravindra Arya; Cristina Barcia Aguilar; James Nicholas Brenton; Jessica L Carpenter; Kevin E Chapman; Justice Clark; Raquel Farias-Moeller; William D Gaillard; Marina Gaínza-Lein; Tracy Glauser; Joshua Goldstein; Howard P Goodkin; Réjean M Guerriero; Yi-Chen Lai; Tiffani McDonough; Mohamad A Mikati; Lindsey A Morgan; Edward Novotny; Eric Payne; Katrina Peariso; Juan Piantino; Adam Ostendorf; Tristan T Sands; Kumar Sannagowdara; Robert C Tasker; Dimtry Tchapyjnikov; Alexis A Topjian; Alejandra Vasquez; Mark S Wainwright; Angus Wilfong; Kowryn Williams; Tobias Loddenkemper Journal: Neurology Date: 2020-07-01 Impact factor: 9.910
Authors: Kyle H Bennett; Suresh S Pujar; Marina M Martinos; Christopher A Clark; Michael Yoong; Rod C Scott; Richard F M Chin Journal: Epilepsy Behav Date: 2020-06-08 Impact factor: 2.937
Authors: Marina Gaínza-Lein; Cristina Barcia Aguilar; Juan Piantino; Kevin E Chapman; Iván Sánchez Fernández; Marta Amengual-Gual; Anne Anderson; Brian Appavu; Ravindra Arya; James Nicholas Brenton; Jessica L Carpenter; Justice Clark; Raquel Farias-Moeller; William D Gaillard; Tracy A Glauser; Joshua L Goldstein; Howard P Goodkin; Linda Huh; Robert Kahoud; Kush Kapur; Yi-Chen Lai; Tiffani L McDonough; Mohamad A Mikati; Lindsey A Morgan; Anuranjita Nayak; Edward Novotny; Adam P Ostendorf; Eric T Payne; Katrina Peariso; Latania Reece; James Riviello; Kumar Sannagowdara; Tristan T Sands; Theodore Sheehan; Robert C Tasker; Dmitry Tchapyjnikov; Alejandra Vasquez; Mark S Wainwright; Angus Wilfong; Korwyn Williams; Bo Zhang; Tobias Loddenkemper Journal: Epilepsia Date: 2021-07-12 Impact factor: 6.740