1,415 results match your criteria Sudden Unexpected Death in Epilepsy


Discussion of the Brazilian neurologists about sudden unexpected death in epilepsy.

Rev Assoc Med Bras (1992) 2022 May;68(5):675-679

Escola Paulista de Medicina/Universidade Federal de São Paulo, Disciplina de Neurologia Experimental - São Paulo (SP), Brazil.

Objective: This study aimed to evaluate the concept of health professionals affiliated with the Brazilian League of Epilepsy on whether or not to inform patients about the risk factors related to the occurrence of sudden unexpected death in epilepsy.

Methods: A descriptive research of inquiry was conducted with direct survey on the Brazilian neurologist's view, regarding medical behavior in the health area to report or not about the risk of sudden unexpected death in epilepsy. Data collection consisted of a structured questionnaire available online. Read More

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Correction to: Ictal neural oscillatory alterations precede sudden unexpected death in epilepsy.

Authors:

Brain Commun 2022 13;4(3):fcac121. Epub 2022 May 13.

[This corrects the article DOI: 10.1093/braincomms/fcac073.]. Read More

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Pathogenicity Assignment of Variants in Genes Associated With Cardiac Channelopathies Evolve Toward Diagnostic Uncertainty.

Circ Genom Precis Med 2022 May 11:101161CIRCGEN121003491. Epub 2022 May 11.

Division of Pediatric Cardiology, Department of Pediatrics, Duke University School of Medicine, Durham, NC. (M.B.R., I.M.L., A.P.L.).

Background: Accurately determining variant pathogenicity is critical in the diagnosis of cardiac channelopathies; however, it remains unknown how variant pathogenicity status changes over time. Our aim is to use a comprehensive analysis of ClinVar to understand mutability of variant evaluation in channelopathy-associated genes to inform clinical decision-making around variant calling.

Methods: We identified 10 genes () strongly associated with cardiac channelopathies, as well as 3 comparison gene sets (disputed long QT syndrome, sudden unexpected death in epilepsy, and all ClinVar). Read More

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The recovery position for maintenance of adequate ventilation and the prevention of cardiac arrest: A systematic review.

Resusc Plus 2022 Jun 29;10:100236. Epub 2022 Apr 29.

Department of Emergency Medicine, University of Virginia, United States of America.

Aim: To conduct a systematic review of the use of the recovery position in adults and children with non-traumatic decreased levels of responsiveness changes outcomes in comparison with other positioning strategies.

Methods: We searched Medline (Ovid), Embase, Cochrane Library, CINAHL, medRxiv and Google Scholar from inception to 15 March 2021 for studies involving adults and children in an out-of-hospital, first aid setting who had reduced levels of responsiveness of non-traumatic aetiology but did not require resuscitative interventions. We used the ROBINS-I tool to assess risk of bias and GRADE methodology to determine the certainty of evidence. Read More

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Life-span characterization of epilepsy and comorbidities in Dravet syndrome mice carrying a targeted deletion of exon 1 of the Scn1a gene.

Exp Neurol 2022 Apr 26;354:114090. Epub 2022 Apr 26.

Department of Physiology and Medical Physics, RCSI University of Medicine and Health Sciences, Dublin, Ireland; FutureNeuro SFI Research Centre, RCSI University of Medicine and Health Sciences, Dublin, Ireland. Electronic address:

Objective: Dravet Syndrome (DS) is a catastrophic form of paediatric epilepsy associated with multiple comorbidities mainly caused by mutations in the SCN1A gene. DS progresses in three different phases termed febrile, worsening and stabilization stage. Mice that are haploinsufficient for Scn1a faithfully model each stage of DS, although various aspects have not been fully described, including the temporal appearance and sex differences of the epilepsy and comorbidities. Read More

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TAU ablation in excitatory neurons and postnatal TAU knockdown reduce epilepsy, SUDEP, and autism behaviors in a Dravet syndrome model.

Sci Transl Med 2022 Apr 27;14(642):eabm5527. Epub 2022 Apr 27.

Gladstone Institute of Neurological Disease, Gladstone Institutes, San Francisco, CA 94158, USA.

Intracellular accumulation of TAU aggregates is a hallmark of several neurodegenerative diseases. However, global genetic reduction of TAU is beneficial also in models of other brain disorders that lack such TAU pathology, suggesting a pathogenic role of nonaggregated TAU. Here, conditional ablation of TAU in excitatory, but not inhibitory, neurons reduced epilepsy, sudden unexpected death in epilepsy, overactivation of the phosphoinositide 3-kinase-AKT-mammalian target of rapamycin pathway, brain overgrowth (megalencephaly), and autism-like behaviors in a mouse model of Dravet syndrome, a severe epileptic encephalopathy of early childhood. Read More

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Ictal neural oscillatory alterations precede sudden unexpected death in epilepsy.

Brain Commun 2022 25;4(2):fcac073. Epub 2022 Mar 25.

Department of Cell Biology and Physiology, University of North Carolina, Chapel Hill, NC, USA.

Sudden unexpected death in epilepsy is the most catastrophic outcome of epilepsy. Each year there are as many as 1.65 cases of such death for every 1000 individuals with epilepsy. Read More

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New inroads into the brain circuits and network dynamics behind sudden unexpected death in epilepsy.

Brain Commun 2022 13;4(2):fcac097. Epub 2022 Apr 13.

Simons Initiative for the Developing Brain, Patrick Wild Centre, Centre for Discovery Brain Sciences, University of Edinburgh, Edinburgh EH8 9XD, UK.

This scientific commentary refers to 'Hyperexcitable superior colliculus and fatal brainstem spreading depolarization in a model of sudden unexpected death in epilepsy' by Cain . (https://doi.org/10. Read More

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Hyperexcitable superior colliculus and fatal brainstem spreading depolarization in a model of Sudden Unexpected Death in Epilepsy.

Brain Commun 2022 19;4(2):fcac006. Epub 2022 Jan 19.

Djavad Mowafaghian Centre for Brain Health, University of British Columbia, Vancouver, Canada.

Cardiorespiratory arrest and death in mouse models of sudden unexpected death in epilepsy occur when spreading depolarization is triggered by cortical seizures and then propagates to the brainstem. However, the critical brain regions and the specific changes required to allow spreading depolarization to propagate to the brainstem under the relatively rare circumstances leading to a fatal seizure are unknown. We previously found that following cortical seizure-inducing electrical stimulation, spreading depolarization could occur in both the superior and inferior colliculi in Cacna1a mice, but was never observed in wild-type animals or following non-seizure-inducing stimuli in Cacna1a mice. Read More

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January 2022

Dravet syndrome in children-A population-based study.

Epilepsy Res 2022 May 8;182:106922. Epub 2022 Apr 8.

Department of Pediatrics, Institute of Clinical Sciences, Sahlgrenska Academy, University of Gothenburg, 405 30 Gothenburg, Sweden; Queen Silvia Children's Hospital, Sahlgrenska University Hospital, Member of the ERN EpiCARE, 413 45 Gothenburg, Sweden.

Objective: The aim was to describe age at diagnosis, cumulative incidence, SCN1A variants, mortality, seizure types and treatments in children with Dravet Syndrome (DS) in Sweden.

Methods: Children diagnosed with DS, born between January 1st 2000 and December 31st 2018 were included in a population-based study. Clinical data, frequency of seizure types and treatments were collected from caregivers and medical records in 42 children. Read More

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The role of monoaminergic neurons in modulating respiration during sleep and the connection with SUDEP.

Biomed Pharmacother 2022 Apr 19;150:112983. Epub 2022 Apr 19.

Department of Anesthesiology, The Fourth Clinical School of Medicine, Zhejiang Chinese Medical University, Hangzhou 310006, China; Department of Anesthesiology, Affiliated Hangzhou First People's Hospital, Zhejiang University School of Medicine, Hangzhou, 310006, China; Westlake Laboratory of Life Sciences and Biomedicine, Hangzhou 310006, China. Electronic address:

Sudden unexpected death in epilepsy (SUDEP) is the leading cause of death among epilepsy patients, occurring even more frequently in cases with anti-epileptic drug resistance. Despite some advancements in characterizing SUDEP, the underlying mechanism remains incompletely understood. This review summarizes the latest advances in our understanding of the pathogenic mechanisms of SUDEP, in order to identify possible targets for the development of new strategies to prevent SUDEP. Read More

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Leveraging electronic patient diaries in SUDEP risk evaluation.

Epilepsy Res 2022 May 11;182:106924. Epub 2022 Apr 11.

Children's National Research Institute, Children's National Hospital, USA.

Objective: Our aim was to describe the risk factors known to be related to sudden unexpected death in epilepsy (SUDEP) that can be extracted from patients that utilizes an online seizure diary tool (SeizureTracker™).

Method: We conducted a descriptive analysis of SeizureTracker™ users across factors relevant to SUDEP risk. We also compared our app-using cohort to published SUDEP case-control studies. Read More

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Cardiac Structure and Function in Epilepsy Patients with Drug-Resistant Convulsive Seizures.

Case Rep Neurol 2022 Jan-Apr;14(1):88-97. Epub 2022 Mar 10.

Department of Neurology, Stavanger University Hospital, Stavanger, Norway.

High frequency of convulsive seizures and long-lasting epilepsy are associated with an increased risk of sudden unexpected death in epilepsy (SUDEP). Structural changes in the myocardium have been described in SUDEP victims. It is speculated that these changes are secondary to frequent convulsive seizures and may predispose to SUDEP. Read More

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Alterations in aortic vasorelaxation in rats with epilepsy induced by the electrical amygdala kindling model.

Epilepsy Res 2022 May 6;182:106920. Epub 2022 Apr 6.

Department of Physiological Sciences, Federal University of Goiás, Goiânia, Brazil. Electronic address:

Cardiovascular alterations are frequently related to epilepsy in both clinical and experimental models, and have been hypothesized as a potential contributor to sudden unexpected death in epilepsy (SUDEP). Further, the frequency of generalized tonic-clonic seizures (GTCS) is a primary risk factor for SUDEP. Therefore, we aimed to evaluate the vascular response of rats subjected to the electrical amygdala kindling model of epilepsy. Read More

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DEPDC5-related epilepsy: A comprehensive review.

Authors:
Debopam Samanta

Epilepsy Behav 2022 May 14;130:108678. Epub 2022 Apr 14.

Child Neurology Section, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR, United States. Electronic address:

DEPDC5-related epilepsy, caused by pathogenic germline variants(with or without additional somatic variants in the brain) of DEPDC5 (Dishevelled, Egl-10 and Pleckstrin domain-containing protein 5) gene, is a newly discovered predominantly focal epilepsy linked to enhanced mTORC1 pathway. DEPDC5-related epilepsy includes several familial epilepsy syndromes, including familial focal epilepsy with variable foci (FFEVF) and rare sporadic nonlesional focal epilepsy. DEPDC5 has been identified as one of the more common epilepsy genes linked to infantile spasms and sudden unexpected death (SUDEP). Read More

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Evaluation of Heart Rate Variability Parameters During Awake and Sleep in Refractory and Controlled Epileptic Patients.

Int J Gen Med 2022 8;15:3865-3877. Epub 2022 Apr 8.

Department of Internal Medicine, Faculty of Medicine (for Girls), Al-Azhar University, Cairo, Egypt.

Background: Alterations of heart rate variability (HRV) in epileptic patients were the field of interest of several studies for many reasons, particularly the contribution toward sudden unexpected death in epilepsy (SUDEP).

Aim: We aimed at evaluation of autonomic dysfunction in epileptic patients during awake and sleep in addition to studying the association between SUDEP risk with different Holter parameters.

Patients And Methods: The study included eighty epileptic patients (40 controlled epileptic patients and 40 refractory epileptic patients) compared to 30 volunteers as control group. Read More

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Targeted Molecular Strategies for Genetic Neurodevelopmental Disorders: Emerging Lessons from Dravet Syndrome.

Neuroscientist 2022 Apr 13:10738584221088244. Epub 2022 Apr 13.

Department of Pediatrics, Division of Pediatric Neurology, Developmental Medicine and Social Pediatrics, University Hospital of Munich, Ludwig Maximilians University, Munich, Germany.

Dravet syndrome is a severe developmental and epileptic encephalopathy mostly caused by heterozygous mutation of the gene encoding the voltage-gated sodium channel α subunit Na1.1. Multiple seizure types, cognitive deterioration, behavioral disturbances, ataxia, and sudden unexpected death associated with epilepsy are a hallmark of the disease. Read More

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Risk factors for postictal generalized EEG suppression in generalized convulsive seizure: A systematic review and meta-analysis.

Seizure 2022 May 27;98:19-26. Epub 2022 Mar 27.

Department of Neurology, Qilu Hospital of Shandong University, No.107 West Wenhua Road, Jinan, Shandong Province 250012, China. Electronic address:

Background And Purpose: Postictal generalized EEG suppression (PGES) has been suggested as a pathophysiological hallmark for sudden unexpected death in epilepsy (SUDEP). We aimed to characterize the clinical determinants for PGES occurrence after generalized convulsive seizures (GCS).

Methods: We systematically searched Pubmed, Embase and Medline databases up to 30 August 2021. Read More

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Rare sudden unexpected death in epilepsy SCN5A variants cause changes in channel function implicating cardiac arrhythmia as a cause of death.

Epilepsia 2022 Apr 9. Epub 2022 Apr 9.

Florey Institute of Neuroscience and Mental Health, University of Melbourne, Parkville, Victoria, Australia.

Sudden unexpected death in epilepsy (SUDEP) is a leading cause of premature death in epilepsy. The underlying pathological mechanisms are likely to be multifactorial. Cardiac arrhythmia has been suggested as a cause of death in some patients with SUDEP. Read More

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[Current concepts about autonomic dysfunction in patients with epilepsy].

Zh Nevrol Psikhiatr Im S S Korsakova 2022 ;122(3):131-138

Moscow Research and Clinical Center for Neuropsychiatry, Moscow, Russia.

Autonomic dysfunction is frequently observed in people with epilepsy. Ictal and postictal dysautonomia is not only manifestation of autonomic seizures, but could be a life threatening condition in some cases and contribute to sudden unexpected death (SUDEP). Interictal decrease of autonomic activity is associated with duration and severity of epilepsy, it is the most prominent in focal and drug-resistant epilepsy. Read More

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Serious Cardiac Arrhythmias Detected by Subcutaneous Long-term Cardiac Monitors in Patients With Drug-Resistant Epilepsy.

Neurology 2022 05 6;98(19):e1923-e1932. Epub 2022 Apr 6.

From the Department of Neuroscience, Central Clinical School (S.S., Z.L., P.C.-E., N.C.J., M.T., C.F., J.-P.N., P.P., P.K., T.J.O.), Monash University; Department of Neurology (S.S., P.C.-E., N.C.J., M.T., J.-P.N., P.P., P.K., T.J.O.), The Alfred Hospital, Melbourne; Department of Medicine (S.S., P.C.-E., N.C.J., M.T., V.C., R.Y., C.F., J.-P.N., P.K., T.J.O.), The Royal Melbourne Hospital, The University of Melbourne; Departments of Neurology (S.S., F.S., M.T., V.C., R.Y., C.F., J.-P.N., P.P., P.K., T.J.O.) and Cardiology (F.S., P.S.), The Royal Melbourne Hospital, Parkville; Department of Neurology (E.M., U.S.), Monash Medical Centre, Clayton, Australia; Academic Neurology Unit (V.C.), Royal Hallamshire Hospital, University of Sheffield, Division of Neuroscience and Experimental Psychology (V.C.), School of Biological Sciences, University of Manchester, UK; Department of Medicine (P.P.), Austin Hospital, The University of Melbourne; and Bladin-Berkovic Comprehensive Epilepsy Program (P.P.), Austin Health, Heidelberg, Australia.

Background And Objectives: Epilepsy is associated with an increased risk of cardiovascular disease and premature mortality, including sudden unexpected death in epilepsy (SUDEP). Serious cardiac arrythmias might go undetected in routine epilepsy and cardiac investigations.

Methods: This prospective cohort study aimed to detect cardiac arrhythmias in patients with chronic drug-resistant epilepsy (≥5 years duration) using subcutaneous cardiac monitors for a minimum follow-up duration of 12 months. Read More

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Interictal EEG and ECG for SUDEP Risk Assessment: A Retrospective Multicenter Cohort Study.

Front Neurol 2022 18;13:858333. Epub 2022 Mar 18.

Neuroscience Institute, New York University Grossman School of Medicine, New York, NY, United States.

Objective: Sudden unexpected death in epilepsy (SUDEP) is the leading cause of epilepsy-related mortality. Although lots of effort has been made in identifying clinical risk factors for SUDEP in the literature, there are few validated methods to predict individual SUDEP risk. Prolonged postictal EEG suppression (PGES) is a potential SUDEP biomarker, but its occurrence is infrequent and requires epilepsy monitoring unit admission. Read More

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Proteomic differences in hippocampus and cortex of sudden unexplained death in childhood.

Acta Neuropathol 2022 May 25;143(5):585-599. Epub 2022 Mar 25.

Comprehensive Epilepsy Center, Department of Neurology, NYU Langone Health and Grossman School of Medicine, New York, NY, USA.

Sudden unexplained death in childhood (SUDC) is death of a child over 1 year of age that is unexplained after review of clinical history, circumstances of death, and complete autopsy with ancillary testing. Multiple etiologies may cause SUDC. SUDC and sudden unexpected death in epilepsy (SUDEP) share clinical and pathological features, suggesting some similarities in mechanism of death and possible abnormalities in hippocampus and cortex. Read More

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Effect of Lamotrigine on Ouabain-Induced Arrhythmia in Isolated Atria of Guinea Pigs.

Med J Islam Repub Iran 2021 20;35:138. Epub 2021 Oct 20.

Department of Neurosurgery, Hazrat Rasoul Hospital, Iran University of Medical Sciences, Tehran, Iran.

Lamotrigine (LTG) is an antiepileptic drug used in the treatment of seizures, mood disorders, and cognitive problems. The cardiac effects of LTG, such as LTG toxicity and SUEDP, have been studied. This is an in vitro study examining the effect of LTG on isolated atria of guinea pigs. Read More

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October 2021

Effects of Antiarrhythmic Drugs on Antiepileptic Drug Action-A Critical Review of Experimental Findings.

Int J Mol Sci 2022 Mar 7;23(5). Epub 2022 Mar 7.

Independent Unit of Experimental Neuropathophysiology, Department of Toxicology, Medical University of Lublin, 20-090 Lublin, Poland.

Severe cardiac arrhythmias developing in the course of seizures increase the risk of SUDEP (sudden unexpected death in epilepsy). Hence, epilepsy patients with pre-existing arrhythmias should receive appropriate pharmacotherapy. Concomitant treatment with antiarrhythmic and antiseizure medications creates, however, the possibility of drug-drug interactions. Read More

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Understanding the Molecular Mechanisms of Succinic Semialdehyde Dehydrogenase Deficiency (SSADHD): Towards the Development of SSADH-Targeted Medicine.

Int J Mol Sci 2022 Feb 26;23(5). Epub 2022 Feb 26.

F.M. Kirby Neurobiology Center, Boston Children's Hospital, Boston, MA 02115, USA.

Succinic semialdehyde dehydrogenase deficiency (SSADHD) is a rare genetic disorder caused by inefficient metabolic breakdown of the major inhibitory neurotransmitter, γ-aminobutyric acid (GABA). Pathologic brain accumulation of GABA and γ-hydroxybutyrate (GHB), a neuroactive by-product of GABA catabolism, leads to a multitude of molecular abnormalities beginning in early life, culminating in multifaceted clinical presentations including delayed psychomotor development, intellectual disability, hypotonia, and ataxia. Paradoxically, over half of patients with SSADHD also develop epilepsy and face a significant risk of sudden unexpected death in epilepsy (SUDEP). Read More

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February 2022

Hyperekplexia: A Frequent Near Miss in Infants and Young Children.

Neurol India 2022 Jan-Feb;70(1):312-314

Department of Pediatrics, Child Neurology Division, All India Institute of Medical Sciences, New Delhi, India.

Hyperekplexia, an underdiagnosed motor paroxysm of infancy, mimics epilepsy closely. It is hallmarked by episodic and excessive startle response, brief episodes of intense, generalized hypertonia, or stiffness in response to unexpected auditory and/or tactile stimuli right from birth. Though a seemingly benign entity with an excellent prognosis, hyperekplexia has been occasionally associated with recurrent apneas, feeding difficulties, and sudden infant death syndrome (SIDS). Read More

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Sudden Death and Cardiac Arrythmia With Lamotrigine: A Rapid Systematic Review.

Neurology 2022 04 8;98(17):e1748-e1760. Epub 2022 Mar 8.

From the Stichting Epilepsie Instellingen Nederland (SEIN) (J.W.B., J.W.S., R.D.T., M.R.K.), Heemstede; Department of Neurology (J.W.B., R.D.T.), Leiden University Medical Centre, the Netherlands; School of Public Health (N.H., M.R.K.) and Department of Neurosciences (M.R.K.), Université de Montréal, Canada; Department of Neurology (O.D., J.A.F.), New York University Grossman School of Medicine and NYU Langone Health, New York; UCL Queen Square Institute of Neurology (J.W.S., R.D.T.), London; and Chalfont Centre for Epilepsy (J.W.S., R.D.T.), Chalfont St Peter, UK.

Background And Objectives: A recent Food and Drug Administration warning concerning an arrhythmogenic potential of lamotrigine created concern in the neurologic community. This warning was based on in vitro studies, but no clinically relevant risk was considered. This rapid systematic review aims to elucidate the risk of lamotrigine on sudden death or ECG abnormalities. Read More

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Omega-3 Fatty Acids May Prevent Sudden Unexpected Death in Epilepsy in Children.

J Pediatr Pharmacol Ther 2022 9;27(2):198-199. Epub 2022 Feb 9.

Klinikum Landstrasse (JF), Messerli Institute, Vienna, Austria.

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February 2022

Sudden Unexpected Death in Epilepsy (SUDEP): How Do We Prevent This Childhood Tragedy?

J Pediatr Pharmacol Ther 2022 9;27(2):99-101. Epub 2022 Feb 9.

Le Bonheur Comprehensive Epilepsy Program and Neuroscience Institute (BM, JWW), Le Bonheur Children's Hospital, Memphis, TN; Division of Pediatric Neurology (BM, JWW), Department of Pediatrics, University of Tennessee Health Science Center, Memphis, TN.

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February 2022