1,315 results match your criteria Pediatrics Status Epilepticus


Neuroprotective Effects of Lacosamide and Memantine on Hyperoxic Brain Injury in Rats.

Neurochem Res 2020 May 22. Epub 2020 May 22.

Division of Pediatric Neurology, Department of Pediatrics, Faculty of Medicine, Dokuz Eylul University, Izmir, Turkey.

In neonates supraphysiological oxygen therapy has been demonstrated to cause neuronal death in hippocampus, prefrontal cortex, parietal cortex, and retrosplenial cortex. There is a need for the detection of novel neuroprotective drugs. Neuroprotective effects of lacosamide or memantine have been demonstrated in adult patients with ischemia, trauma and status epilepticus. Read More

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http://dx.doi.org/10.1007/s11064-020-03056-5DOI Listing

The association of patient weight and dose of fosphenytoin, levetiracetam, and valproic acid with treatment success in status epilepticus.

Epilepsia 2020 May 18. Epub 2020 May 18.

Department of Experimental and Clinical Pharmacology and Center for Orphan Drug Research, College of Pharmacy, University of Minnesota, Minneapolis, Minnesota.

The Established Status Epilepticus Treatment Trial was a blinded, comparative-effectiveness study of fosphenytoin, levetiracetam, and valproic acid in benzodiazepine-refractory status epilepticus. The primary outcome was clinical seizure cessation and increased responsiveness without additional anticonvulsant medications. Weight-based dosing was capped at 75 kg. Read More

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http://dx.doi.org/10.1111/epi.16534DOI Listing

Kawasaki disease presented with status epilepticus and diffusion MRI abnormalities in the subcortical white matter.

Turk J Pediatr 2020 ;62(2):315-319

Department of Pediatrics, St. Marianna University School of Medicine, Kawasaki, Kanagawa, Japan.

Background: Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and young children. Encephalitis/encephalopathy is an extremely rare complication of KD.

Case: A previously healthy 8-month-old Japanese boy had a prolonged seizure after febrile illness for one day. Read More

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http://dx.doi.org/10.24953/turkjped.2020.02.020DOI Listing
January 2020

Levetiracetam for Benzodiazepine-Refractory Status Epilepticus.

Indian J Pediatr 2020 May 15. Epub 2020 May 15.

Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

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http://dx.doi.org/10.1007/s12098-020-03341-9DOI Listing

Variable treatment response in a patient with pyridoxal phosphate oxidase (PNPO) deficiency- understanding the paradox.

Epilepsy Behav Rep 2020 24;14:100357. Epub 2020 Mar 24.

Department of Paediatrics, Aster Malabar Institute of Medical Sciences, Kozhikode, Kerala, India.

A 6-year-old girl presented with history of infantile onset epileptic encephalopathy and developmental delay. She had polymorphic seizures that were refractory to regular anti-seizure medication. Incomplete control of seizures was achieved on starting pyridoxine, riboflavin and thiamine. Read More

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http://dx.doi.org/10.1016/j.ebr.2020.100357DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7210397PMC

Intramuscular Versus Buccal Midazolam for Pediatric Seizures: A Randomized Double-Blinded Trial.

Pediatr Neurol 2020 Mar 16. Epub 2020 Mar 16.

Department of Pediatrics, Division of Pediatric Emergency Medicine, Hamad Medical Corporation, Doha, Qatar.

Background: We compared the efficacy and safety of intramuscular with buccal midazolam as first-line treatment for active seizures in children brought to the emergency department.

Methods: In a double-blind, double-dummy randomized trial, patients with an active seizure lasting more than five minutes received blinded treatments on arrival. We employed deferred consent. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2020.03.011DOI Listing

Transcranial photobiomodulation attenuates pentylenetetrazole-induced status epilepticus in peripubertal rats.

J Biophotonics 2020 May 3:e202000095. Epub 2020 May 3.

Department of Pediatrics, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan.

Convulsive status epilepticus is the most common neurological emergency in children. Transcranial photobiomodulation (tPBM) reverses elevated rodent neurotransmitters after status epilepticus (SE) yet whether tPBM can attenuate seizure behaviors remains unknown. Here, we applied near-infrared laser at wavelength 808 nm transcranially to peripubertal Sprague-Dawley rats prior to pentylenetetrazole (PTZ) injection. Read More

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http://dx.doi.org/10.1002/jbio.202000095DOI Listing

Pathogenic variants in KPTN gene identified by Clinical Whole-Genome Sequencing.

Cold Spring Harb Mol Case Stud 2020 May 1. Epub 2020 May 1.

Center for Pediatric Genomic Medicine, Children's Mercy Hospital, Kansas City, MO, USA.

Status epilepticus is not rare in critically ill intensive care unit patients, but its diagnosis is often delayed or missed. The mortality for convulsive status epilepticus is dependent on the underlying aetiologies and the age of the patients, and thus varies from study to study. In this context, effective molecular diagnosis in a pediatric patient with a genetically heterogeneous phenotype is essential. Read More

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http://dx.doi.org/10.1101/mcs.a003970DOI Listing

TRPV1 Contributes to the Neuroprotective Effect of Dexmedetomidine in Pilocarpine-Induced Status Epilepticus Juvenile Rats.

Biomed Res Int 2020 7;2020:7623635. Epub 2020 Apr 7.

Pediatric Research Institute; Ministry of Education Key Laboratory of Child Development and Disorders, National Clinical Research Center for Child Health and Disorders (Chongqing), China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Children's Hospital of Chongqing Medical University, Chongqing 400014, China.

To investigate the antiepileptic and neuroprotective effects of dexmedetomidine (Dex) in pilocarpine- (Pilo-) induced status epilepticus (SE) juvenile rats, rats were randomly assigned to the following six groups ( = 20): normal, normal+Dex, SE, SE+Cap, SE+Dex, and SE+Dex+Cap. The rats were treated with either diazepam (i.p. Read More

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http://dx.doi.org/10.1155/2020/7623635DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7168755PMC

Therapeutic hypothermia for pediatric refractory status epilepticus May Ameliorate post-status epilepticus epilepsy.

Biomed J 2020 Apr 21. Epub 2020 Apr 21.

Division of Pediatric Neurology, Department of Pediatrics at Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan. Electronic address:

Background: To compare the clinical characteristics and outcomes of pediatric patients with refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE) who received therapeutic hypothermia (TH) plus anticonvulsants or anticonvulsants alone.

Material And Methods: Two-medical referral centers, retrospective cohort study.

Setting: Pediatric Intensive Care Unit (PICU) at Taoyuan Chang Gung Children's hospital and Kaohsiung Chang Gung Memorial Hospital. Read More

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http://dx.doi.org/10.1016/j.bj.2020.04.004DOI Listing

CSF levels of a set of neurotrophic factors (brain-derived neurotrophic factor, nerve growth factor) and neuropeptides (neuropeptide Y, galanin) in epileptic children.

J Clin Neurosci 2020 Apr 20. Epub 2020 Apr 20.

Pediatric Neurology Division, Department of Pediatrics, Ege Children's Hospital, Ege University Medical School, Izmir, Turkey. Electronic address:

This paper aims to investigate the possible roles of a set of neurotrophic factors (brain-derived neurotrophic factor-BDNF, nerve growth factor-NGF) and neuropeptides (neuropeptide Y-NPY, and galanin) in children with active epileptogenesis. The cerebrospinal fluid (CSF) levels of BDNF, NPY, NGF and galanin were measured with enzyme-linked immunosorbent assays in epileptic children (n = 73) and controls (n = 64). There were no significant alterations in the CSF levels of BDNF, NPY and NGF in epileptic children with active clinical seizures compared with the levels of controls. Read More

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http://dx.doi.org/10.1016/j.jocn.2020.04.064DOI Listing

Praxis-induced reflex seizures in two Japanese cases with ring chromosome 20 syndrome.

Epileptic Disord 2020 Apr;22(2):214-218

Department of Paediatrics, Jichi Medical University, Shimotsuke, Japan.

Ring chromosome 20 syndrome is an epileptic and neurodevelopmental encephalopathy that occurs in children, characterised by a triad of refractory frontal lobe seizures, recurrent non-convulsive status epilepticus and frontal lobe-dominant paroxysmal discharges. However, details of other clinical features associated with ring chromosome 20 syndrome remain unknown. Here, we report two patients with ring chromosome 20 syndrome who had praxis-induced reflex seizures. Read More

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http://dx.doi.org/10.1684/epd.2020.1146DOI Listing

Corrigendum to "The role of necroptosis in status epilepticus-induced brain injury in juvenile rats" [Epilepsy Behav, 75 (2017): 134-142].

Epilepsy Behav 2020 Jun 13;107:107059. Epub 2020 Apr 13.

Department of Pediatrics, West China Second University Hospital, Sichuan University, Chengdu 610041, Sichuan, China; Key Laboratory of Obstetric & Gynecologic and Pediatric Diseases and Birth Defects of Ministry of Education, Sichuan University, Chengdu 610041, Sichuan, China. Electronic address:

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http://dx.doi.org/10.1016/j.yebeh.2020.107059DOI Listing

High-power, frontal-dominant ripples in absence status epilepticus during childhood.

Clin Neurophysiol 2020 Jun 19;131(6):1204-1209. Epub 2020 Mar 19.

Division of Neurology, Saitama Children's Medical Center, 2-1 Shin-toshin, Chuou-ku, Saitama-city, Saitama 330-8777, Japan; Department of Pediatrics, Jikei University School of Medicine, 3-19-18 Nishi-shinbashi, Minato-ku, Tokyo 105-8471, Japan.

Objective: Absence status epilepticus (ASE) is a form of non-convulsive status epilepticus characterized by ongoing or intermittent epileptic activity accompanied by behavioral and cognitive changes. Herein, we assessed high-frequency oscillations in the ripple band in patients with ASE and typical absence seizures.

Methods: We enrolled five patients with ASE, 26 patients with childhood absence epilepsy (CAE), and 15 patients with juvenile absence epilepsy (JAE). Read More

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http://dx.doi.org/10.1016/j.clinph.2020.02.024DOI Listing

Clinical characteristics and epilepsy in genomic imprinting disorders: Angelman syndrome and Prader-Willi syndrome.

Ci Ji Yi Xue Za Zhi 2019 Apr-Jun;32(2):137-144. Epub 2019 Oct 31.

Department of Pediatrics, Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation, New Taipei, Taiwan.

Angelman syndrome (AS) and Prader-Willi syndrome (PWS) are considered sister imprinting disorders. Although both AS and PWS congenital neurodevelopmental disorders have chromosome 15q11.3-q13 dysfunction, their molecular mechanisms differ owing to genomic imprinting, which results in different parent-of-the-origin gene expressions. Read More

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http://dx.doi.org/10.4103/tcmj.tcmj_103_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7137370PMC
October 2019

Reply to Dr. Capovilla on "Reply to the article "Management of status epilepticus in adults. Position paper of the Italian League Against Epilepsy".

Epilepsy Behav 2020 Jun 6;107:107048. Epub 2020 Apr 6.

IRCCS Istituto delle Scienze Neurologiche, Bellaria Hospital, Bologna, Italy; Department of Biomedical and Neuromotor Sciences, University of Bologna, Italy.

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http://dx.doi.org/10.1016/j.yebeh.2020.107048DOI Listing

A reappraisal of the value of video-EEG recording in the emergency department.

Expert Rev Neurother 2020 May 4;20(5):459-475. Epub 2020 Apr 4.

Chair of Pediatrics, Child Neurology, NESMOS Department, Faculty of Medicine and Psychology, Sapienza University, c/o Sant'Andrea Hospital, Rome, Italy.

: Some neurologic conditions that can quickly and with low costs be recognized, classified and treated thanks to the availability of an EEG recording in an emergency setting. However, although considered a cheap, not invasive, highly accurate diagnostic investigation, still today, an EEG recording in emergency, in real time during the event paroxysmal ictal phase, is not yet been become a routine.: This review will cover the role and utility of EEG recording in the emergency setting, both in emergency department and intensive care unit, in adult and pediatric age, in people admitted for status epilepticus (convulsive or non-convulsive), paroxysmal non-epileptic events, or other conditions/diseases presenting with mental status changes. Read More

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http://dx.doi.org/10.1080/14737175.2020.1747435DOI Listing

Efficacy of levetiracetam, fosphenytoin, and valproate for established status epilepticus by age group (ESETT): a double-blind, responsive-adaptive, randomised controlled trial.

Lancet 2020 04 20;395(10231):1217-1224. Epub 2020 Mar 20.

Department of Emergency Medicine, Neuro Emergencies Research, University of Michigan, Ann Arbor, MI, USA. Electronic address:

Background: Benzodiazepine-refractory, or established, status epilepticus is thought to be of similar pathophysiology in children and adults, but differences in underlying aetiology and pharmacodynamics might differentially affect response to therapy. In the Established Status Epilepticus Treatment Trial (ESETT) we compared the efficacy and safety of levetiracetam, fosphenytoin, and valproate in established status epilepticus, and here we describe our results after extending enrolment in children to compare outcomes in three age groups.

Methods: In this multicentre, double-blind, response-adaptive, randomised controlled trial, we recruited patients from 58 hospital emergency departments across the USA. Read More

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http://dx.doi.org/10.1016/S0140-6736(20)30611-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7241415PMC

Refractory and Super-refractory Status Epilepticus.

Indian Pediatr 2020 Mar;57(3):239-253

Comprehensive Epilepsy Center, Division of Neurology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio; USA. Correspondence to: Dr Ravindra Arya, Division of Neurology, Cincinnati Children's Hospital Medical Center, MLC 2015, 3333 Burnet Avenue, Cincinnati, Ohio, 45229 USA.

Context: Refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE) are neurological emergencies with considerable mortality and morbidity. In this paper, we provide an overview of causes, evaluation, treatment, and consequences of RSE and SRSE, reflecting the lack of high-quality evidence to inform therapeutic approach.

Sources: This is a narrative review based on personal practice and experience. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7224023PMC

Comparison of Phenytoin, Valproate and Levetiracetam in Pediatric Convulsive Status Epilepticus: A Randomized Double-blind Controlled Clinical Trial.

Indian Pediatr 2020 Mar;57(3):222-227

Division of Pediatric Critical Care, Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India.

Objective: To compare the efficacy of phenytoin, valproate, and levetiracetam in the management of pediatric convulsive status epilepticus.

Design: Randomized double-blind controlled clinical trial.

Setting: Pediatric critical care division in a tertiary care institute from June, 2016 to December, 2018. Read More

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Efficacy, Tolerability and Serum Phenytoin Levels after Intravenous Fosphenytoin Loading Dose in Children with Status Epilepticus.

Indian Pediatr 2020 Mar;57(3):218-221

Pediatric Neurology Unit, Department of Pediatrics, Bharati Vidyapeeth Deemed University Medical College, Pune, India.

Objective: To evaluate the efficacy and tolerability of intravenous fosphenytoin in children with status epilepticus, and resulting serum total phenytoin levels.

Methods: In this prospective study, 51 children aged less than 18 years received intravenous loading dose of fosphenytoin (18-20 mg/kg). Serum total phenytoin levels were estimated at 90 -100 minutes. Read More

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March 2020
1.014 Impact Factor

Clinical Profile and Short-term Outcome of Pediatric Status Epilepticus at a Tertiary-care Center in Northern India.

Indian Pediatr 2020 Mar;57(3):213-217

Division of Pediatric Neurology, Department of Pediatrics, Lady Hardinge Medical College and associated Kalawati Saran Children Hospital, New Delhi, India.

Objective: To assess clinical profile and short term treatment outcomes of pediatric status epilepticus (SE) at a tertiary-care center in northern India.

Methods: Prospective cohort study enrolled children aged 1 month to 18 years presenting with SE to the emergency department. Enrolled children (109) were treated as per hospital protocols. Read More

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March 2020
1.014 Impact Factor

Fosphenytoin in Status Epilepticus: The Ice Needs to be Broken.

Indian Pediatr 2020 Mar;57(3):209-210

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

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Clinical Profile and Short-term Outcome of Pediatric Status Epilepticus.

Authors:
Mahesh Kamate

Indian Pediatr 2020 Mar;57(3):207-208

Child Development and Pediatric Neurology Division, Department of Pediatrics, KAHER's JN Medical College, Belgaum, Karnataka 590010.

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Extinguishing Febrile Infection-Related Epilepsy Syndrome: Pipe Dream or Reality?

Semin Neurol 2020 Apr 17;40(2):263-272. Epub 2020 Mar 17.

Division of Epilepsy, Department of Neurology, Mayo Clinic, Rochester, Minnesota.

Febrile infection-related epilepsy syndrome (FIRES) is a rare and devastating epileptic encephalopathy with historically abysmal neurocognitive outcomes, including a high incidence of mortality. It tends to affect children and young adults and is characterized by superrefractory status epilepticus following a recent febrile illness. Growing evidence suggests a heterogeneous etiology resulting in fulminant nonantibody-mediated neuroinflammation. Read More

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http://dx.doi.org/10.1055/s-0040-1708503DOI Listing

Dravet Syndrome: A Review of Current Management.

Pediatr Neurol 2020 Jun 31;107:28-40. Epub 2020 Jan 31.

Division of Pediatric Neurology, Department of Pediatrics & Neurology, University of Tennessee Health Science Center, Le Bonheur Children's Hospital, Memphis, Tennessee.

Dravet syndrome is a debilitating epileptic encephalopathy of childhood with few treatment options available in the United States before 2018. In the modern era, new genetic testing options will allow diagnosis closer to disease onset. Three new medicines-stiripentol, cannabidiol, and fenfluramine-have documented efficacy and safety as adjunctive therapies for treating pharmacoresistant Dravet syndrome. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2020.01.005DOI Listing

Post-Traumatic West Syndrome due to Abusive Head Trauma in Two Infants with Different Brain Imaging Findings.

Tohoku J Exp Med 2020 03;250(3):167-171

Department of Pediatrics, Nagoya City West Medical Center.

Abusive head trauma (AHT), commonly known as shaken baby syndrome, is a cranial injury of infants and young children. AHT is an important cause of morbidity and mortality in young children, particularly those younger than 12 months of age. We describe two patients who developed West syndrome, which is a severe epilepsy syndrome composed of the triad of infantile spasms, hypsarrhythmia on electroencephalography, and developmental arrest or regression, possibly attributable to AHT. Read More

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http://dx.doi.org/10.1620/tjem.250.167DOI Listing

Treatment Outcomes of Pediatric Status Epilepticus in a Tertiary Pediatric Intensive Care Unit.

Pediatr Emerg Care 2020 Mar 3. Epub 2020 Mar 3.

Department of Pediatric Neurology, Tepecik Training and Investigation Hospital, Izmir, Turkey.

Objective: Status epilepticus is associated with high rates of morbidity and mortality; thus, early diagnosis and proper treatment are crucial. We aimed to study the etiology, clinical features, and treatment among pediatric patients with convulsive status epilepticus.

Methods: The medical records of 100 patients were retrospectively obtained from pediatric intensive care unit. Read More

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http://dx.doi.org/10.1097/PEC.0000000000001914DOI Listing

Cardiac dysregulation following intrahippocampal kainate-induced status epilepticus.

Sci Rep 2020 Mar 4;10(1):4043. Epub 2020 Mar 4.

Department of Neuroscience, Baylor College of Medicine, Houston, TX, USA.

Status epilepticus (SE) is a prevalent disorder associated with significant morbidity, including the development of epilepsy and mortality. Cardiac arrhythmias (i.e. Read More

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http://dx.doi.org/10.1038/s41598-020-60324-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7055295PMC

A detailed description of the phenotypic spectrum of North Sea Progressive Myoclonus Epilepsy in a large cohort of seventeen patients.

Parkinsonism Relat Disord 2020 03 18;72:44-48. Epub 2020 Feb 18.

Department of Neurology, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9700 RB, Groningen, the Netherlands; Department of Genetics, University of Groningen, University Medical Center Groningen, Hanzeplein 1, 9700 RB, Groningen, the Netherlands; Pediatrics, Department of Clinical Sciences, Lund University, Sweden. Electronic address:

Introduction: In 2011, a homozygous mutation in GOSR2 (c.430G > T; p. Gly144Trp) was reported as a novel cause of Progressive Myoclonus Epilepsy (PME) with early-onset ataxia. Read More

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http://dx.doi.org/10.1016/j.parkreldis.2020.02.005DOI Listing

[Clinical phenotypes of epilepsy associated with GABRA1 gene variants].

Zhonghua Er Ke Za Zhi 2020 Feb;58(2):118-122

Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.

To summarize the clinical phenotypes of epilepsy in patients with GABRA1 gene variants. A total of 11 epileptic patients (4 boys and 7 girls) who were treated in the Department of Pediatrics, Peking University First Hospital from March 2016 to July 2019 and detected with GABRA1 gene heterozygous pathogenic variants by targeted next-generation sequencing were enrolled. The features of clinical manifestations, electroencephalogram (EEG), and neuroimaging were analyzed retrospectively. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2020.02.010DOI Listing
February 2020

Consensus-based statements for the management of mitochondrial stroke-like episodes.

Wellcome Open Res 2019 13;4:201. Epub 2019 Dec 13.

Wellcome Centre for Mitochondrial Research, Newcastle University, UK, Newcastle upon Tyne, Tyne and Wear, NE2 4HH, UK.

Focal-onset seizures and encephalopathy are prominent features of a stroke-like episode, which is a severe neurological manifestation associated with subtypes of mitochondrial disease. Despite more than 30 years of research, the acute treatment of stroke-like episodes remains controversial. We used the modified Delphi process to harness the clinical expertise of a group of mitochondrial disease specialists from five European countries to produce consensus guidance for the acute management of stroke-like episodes and commonly associated complications. Read More

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http://dx.doi.org/10.12688/wellcomeopenres.15599.1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7014928PMC
December 2019

Clinical Effectiveness of Levetiracetam Compared to Fosphenytoin in the Treatment of Benzodiazepine Refractory Convulsive Status Epilepticus.

Indian J Pediatr 2020 Feb 22. Epub 2020 Feb 22.

Department of Research & Academics, Mehta Multi-speciality Hospitals, Chennai, Tamil Nadu, India.

Objectives: To determine whether levetiracetam is an alternative to fosphenytoin to control Benzodiazepine Refractory Status Epilepticus (BRSE) in pediatric population and also to compare the acute drug related side-effects and ventilation requirement among the both arms of anti-epileptic drug therapy.

Methods: All consecutive children admitted with BRSE were randomized to group A, who received fosphenytoin at 20 mg/kg phenytoin equivalents (PE) dose and group B who received levetiracetam at 40 mg/kg over 10 min. Time to terminate seizure (response latency) was measured. Read More

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http://dx.doi.org/10.1007/s12098-020-03221-2DOI Listing
February 2020

Focal cortical hypermetabolism in atypical benign rolandic epilepsy.

Epilepsy Res 2020 Mar 10;161:106288. Epub 2020 Feb 10.

Department of Neurology, The Royal Children's Hospital, 50 Flemington Road, Parkville, Victoria, 3052, Australia; Developmental Brain Imaging and Neuroscience Research Groups, Murdoch Children's Research Institute, 50 Flemington Road, Parkville, Victoria, 3052, Australia; Department of Pediatrics, The University of Melbourne, Grattan Street, Parkville, 3010, Australia. Electronic address:

Objective: Atypical benign rolandic epilepsy (BRE) is an underrecognized and poorly understood manifestation of a common epileptic syndrome. Most consider it a focal epileptic encephalopathy in which frequent, interictal, centrotemporal spikes lead to negative motor seizures and interfere with motor and sometimes speech and cognitive abilities. We observed focal cortical hypermetabolism on PET in three children with atypical BRE and investigated the spatial and temporal relationship with their centrotemporal spikes. Read More

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http://dx.doi.org/10.1016/j.eplepsyres.2020.106288DOI Listing

Changing Landscape of Dravet Syndrome Management: An Overview.

Authors:
Debopam Samanta

Neuropediatrics 2020 04 20;51(2):135-145. Epub 2020 Feb 20.

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

Dravet syndrome (DS), previously known as severe myoclonic epilepsy of infancy, is a severe developmental and epileptic encephalopathy caused by loss-of-function mutations in one copy of (haploinsufficiency), located on chromosome 2q24, with decreased function of Nav1.1 sodium channels in GABAergic inhibitory interneurons. Pharmacoresistant seizures in DS start in the infancy in the form of hemiclonic febrile status epilepticus. Read More

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http://dx.doi.org/10.1055/s-0040-1701694DOI Listing

Development of a model to predict electroencephalographic seizures in critically ill children.

Epilepsia 2020 Mar 20;61(3):498-508. Epub 2020 Feb 20.

Department of Pediatrics (Division of Neurology), Children's Hospital of Philadelphia, Philadelphia, PA, USA.

Objective: Electroencephalographic seizures (ESs) are common in encephalopathic critically ill children, but ES identification with continuous electroencephalography (EEG) monitoring (CEEG) is resource-intense. We aimed to develop an ES prediction model that would enable clinicians to stratify patients by ES risk and optimally target limited CEEG resources. We aimed to determine whether incorporating data from a screening EEG yielded better performance characteristics than models using clinical variables alone. Read More

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http://dx.doi.org/10.1111/epi.16448DOI Listing

Surgical management of pediatric patients with encephalopathy due to electrical status epilepticus during sleep (ESES).

Epileptic Disord 2020 Feb;22(1):39-54

Division of Pediatric Neurology, Nicklaus Children's Hospital, Miami, USA.

ESES is a developmental epileptic disorder directly responsible for progressive encephalopathy and neurocognitive regression. The natural history, indications for surgical intervention, and predictors for favorable seizure and neuropsychological outcome remain unclear. We performed a retrospective review of children who underwent resective or disconnective surgery for ESES between January 2009 and July 2016 at a large tertiary pediatric center. Read More

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http://dx.doi.org/10.1684/epd.2020.1129DOI Listing
February 2020
0.899 Impact Factor

Epileptic encephalopathy with features of rapid-onset dystonia Parkinsonism and alternating hemiplegia of childhood: a novel combination phenotype associated with ATP1A3 mutation.

Epileptic Disord 2020 Feb;22(1):103-109

Department of Pediatrics, Duke University Medical Center.

Mutations in ATP1A3 have been found to cause rapid-onset dystonia Parkinsonism, alternating hemiplegia of childhood, epileptic encephalopathy and other syndromes. We report a four-year, nine-month-old boy with episodes of frequent and recurrent status epilepticus, who first began having generalized tonic-clonic seizures at four months of age. Development was normal until the age of four months, and markedly slowed down after the onset of seizures. Read More

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http://dx.doi.org/10.1684/epd.2020.1127DOI Listing
February 2020

Status Epilepticus as a Consequence of Hemophagocytic Lymphohistiocytosis in a Previously Healthy Infant.

Cureus 2020 Jan 30;12(1):e6824. Epub 2020 Jan 30.

Pediatric Intensive Care Unit, King Saud University, Riyadh, SAU.

Hemophagocytic lymphohistiocytosis (HLH) is a rare and fatal entity with an incidence rate of 1.2 cases per million people per year. HLH is explained as a highly destructive inflammatory consequence of rampant hypercytokinemia due to excessive lymphocyte-mediated activation of macrophages and histiocytes. Read More

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http://dx.doi.org/10.7759/cureus.6824DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6996086PMC
January 2020

Usefulness of Continuous Glucose Monitoring for Prevention and Early Detection of Hypoglycemia Caused by a Ketogenic Diet and Late Dumping Syndrome.

Pediatr Neurol 2020 Apr 26;105:65-66. Epub 2019 Dec 26.

Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.12.002DOI Listing

Encephalopathy related to status epilepticus during slow sleep (ESES) as atypical evolution of Panayiotopoulos syndrome: an EEG and neuropsychological study.

Epileptic Disord 2020 Feb;22(1):67-72

Department of Pediatrics, Tokyo Women's Medical University, Tokyo.

We report two patients with Panayiotopoulos syndrome (PS) who developed encephalopathy related to status epilepticus during slow sleep (ESES) at the peak of their clinical course. Clinical charts and EEG data were reviewed. The patients exhibited nocturnal autonomic seizures and occipital EEG foci, the latter of which later evolved into multifocal EEG foci with synchronous frontopolar and occipital spikes (Fp-O EEG foci), and finally into continuous spikes-waves during sleep (CSWS; spike-wave index >85% based on whole-night sleep recording) at eight years and seven years of age, respectively. Read More

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http://dx.doi.org/10.1684/epd.2020.1128DOI Listing
February 2020

Ketamine Infusion for Super Refractory Status Epilepticus in Alternating Hemiplegia of Childhood.

Authors:
Debopam Samanta

Neuropediatrics 2020 Jun 3;51(3):225-228. Epub 2020 Feb 3.

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

Alternating hemiplegia of childhood (AHC) is a rare neurodevelopmental disorder and characterized by infantile onset hemiplegia involving either side of the body and other paroxysmal spells, including epilepsy. The N-methyl-D-aspartate (NMDA) receptor noncompetitive antagonist ketamine has been proved to be effective terminating status epilepticus (SE) in animal models and human case studies. Less than 50 cases, those use ketamine, have been reported in the management of pediatric SE, but its effectiveness is unknown in patients with AHC. Read More

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http://dx.doi.org/10.1055/s-0039-3402005DOI Listing

Detailed characteristics of acute encephalopathy with biphasic seizures and late reduced diffusion: 18-year data of a single-center consecutive cohort.

J Neurol Sci 2020 Apr 16;411:116684. Epub 2020 Jan 16.

Department of Pediatrics, Kobe University Graduate School of Medicine, Hyogo, Japan.

Objective: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a syndrome characterized by biphasic seizures with impaired consciousness. AESD is rare outside Asia, and consecutive cohort studies are therefore scarce. Herein, we aimed to describe the detailed characteristics of AESD, including clinical course, electroencephalogram data, laboratory data, imaging findings, treatment, and outcomes. Read More

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http://dx.doi.org/10.1016/j.jns.2020.116684DOI Listing

Massive lamotrigine and bupropion overdose resulting in status epilepticus without cardiovascular collapse.

Toxicol Commun 2019 22;3(1):102-105. Epub 2019 Dec 22.

Emergency Medicine, Program in Medical Toxicology, Boston Children's Hospital, Boston, MA, USA.

An 18 year-old woman presented to an outside hospital with seizure activity after a massive ingestion of lamotrigine, bupropion, trazodone, buspirone, and possibly isoretinoin. Her initial vital signs were remarkable for tachycardia (120 bpm). She was intubated for airway protection. Read More

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http://dx.doi.org/10.1080/24734306.2019.1699750DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6980321PMC
December 2019

From international guidelines to real-world practice consensus on investigations and management of status epilepticus in adults: A modified Delphi approach.

J Clin Neurosci 2020 Feb 23;72:84-92. Epub 2020 Jan 23.

Department of Clinical Epidemiology and Biostatistics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

Objective: To establish a consensus which is practical and ready-to-use on investigations (ISE) and for management of status epilepticus (MSE) in adults using a modified Delphi approach.

Patients And Methods: A 4-round modified Delphi approach was used. First and second rounds were conducted using Google® survey with structured statements and 6-point Likert scale response. Read More

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http://dx.doi.org/10.1016/j.jocn.2020.01.008DOI Listing
February 2020
1.318 Impact Factor

The mitochondrial epilepsies.

Eur J Paediatr Neurol 2020 Jan 7;24:47-52. Epub 2020 Jan 7.

Wellcome Centre for Mitochondrial Research, Institute of Neuroscience, Newcastle University, Newcastle-Upon-Tyne, NE2 4HH, United Kingdom; Department of Neurology, Royal Victoria Infirmary, Queen Victoria Rd, Newcastle-Upon-Tyne, NE1 4LP, United Kingdom; Institute of Neuroscience, Henry Wellcome Building, Framlington Place, Newcastle University, Newcastle-Upon-Tyne, NE2 4HH, United Kingdom. Electronic address:

Mitochondria are vital organelles within cells that undertake many important metabolic roles, the most significant of which is to generate energy to support organ function. Dysfunction of the mitochondrion can lead to a wide range of clinical features, predominantly affecting organs with a high metabolic demand such as the brain. One of the main neurological manifestations of mitochondrial disease is metabolic epilepsies. Read More

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http://dx.doi.org/10.1016/j.ejpn.2019.12.021DOI Listing
January 2020

Dose-Dependent Behavioral and Antioxidant Effects of Quercetin and Methanolic and Acetonic Extracts from on Several Rat Tissues following Kainic Acid-Induced .

Oxid Med Cell Longev 2019 19;2019:5287507. Epub 2019 Dec 19.

Laboratory of Neuroscience, National Institute of Pediatrics, Mexico City 04530, Mexico.

Kainic acid (KA) has been used to study the neurotoxicity induced after (SE) due to activation of excitatory amino acids with neuronal damage. Medicinal plants can protect against damage caused by KA-induced SE; in particular, organic extracts of and its metabolite quercetin display antioxidant activity and act as hepatoprotective agents. However, it is unknown whether these properties can protect against the hyperexcitability underlying the damage caused by KA-induced SE. Read More

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http://dx.doi.org/10.1155/2019/5287507DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6939434PMC
December 2019

Infant with status epilepticus secondary to systemic lidocaine toxicity from topical application.

BMJ Case Rep 2020 Jan 12;13(1). Epub 2020 Jan 12.

Emergency Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA

A previously healthy 11-month-old infant presented to the emergency department in status epilepticus. There was no clear trigger of her seizure activity which resolved with benzodiazepines and fosphenytoin. On further review, her parents disclosed that she had been prescribed topical 4% lidocaine cream for a groin rash and was ultimately diagnosed with lidocaine toxicity in the emergency department. Read More

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http://dx.doi.org/10.1136/bcr-2019-233119DOI Listing
January 2020

Seizure Characteristics, Outcome, and Risk of Epilepsy in Pediatric Anti-N-Methyl-d-Aspartate Receptor Encephalitis.

Pediatr Neurol 2020 Apr 30;105:35-40. Epub 2019 Nov 30.

Department of Neurology, Children's Hospital of Chongqing Medical University, Chongqing, China; Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China; National Clinical Research Center for Child Health and Disorders, Chongqing, China; China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing, China; Chongqing Key Laboratory of Pediatrics, Chongqing, China. Electronic address:

Background: We identified seizure characteristics, long-term outcome, and predictors of persistent seizures in children with anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis.

Method: Data were analyzed from patients with anti-NMDAR encephalitis who presented with seizures at our center between August 2012 and June 2018.

Results: Sixty-two of 86 patients with anti-NMDAR encephalitis experienced seizures. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.11.011DOI Listing

[Genotype and phenotype of children with KCNA2 gene related developmental and epileptic encephalopathy].

Zhonghua Er Ke Za Zhi 2020 Jan;58(1):35-40

Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.

To investigate the genotype and phenotype of children with KCNA2 gene related developmental and epileptic encephalopathy (DEE). Clinical data including the manifestations and electroencephalogram of 8 children with KCNA2 variants treated in the Department of Pediatrics, Peking University First Hospital from March 2017 to June 2019 were collected and analyzed retrospectively. Among the 8 epileptic patients with KCNA2 variants, 5 were males and 3 were females. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2020.01.009DOI Listing
January 2020