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Neural Regen Res 2019 Mar;14(3):480-484

Department of Neurology, Children's Hospital of Chongqing Medical University; Chongqing International Science and Technology Cooperation Center for Child Development and Disorders; Ministry of Education Key Laboratory of Child Development and Disorders; Key Laboratory of Pediatrics in Chongqing, Chongqing, China.

Status epilepticus has been shown to activate the proliferation of neural stem cells in the hippocampus of the brain, while also causing a large amount of neuronal death, especially in the subgranular zone of the dentate gyrus and the subventricular zone. Simultaneously, proliferating stem cells tend to migrate to areas with obvious damage. Our previous studies have clearly confirmed the effect of sodium valproate on cognitive function in rats with convulsive status epilepticus. Read More

Epileptic Disord 2018 Dec 10. Epub 2018 Dec 10.

Division of Neurology, Department of Pediatrics, Montreal Children's Hospital, McGill University,, Department of Neurology and Neurosurgery, McGill University,, Research Institute of the McGill University Health Centre.

Hemiconvulsion-Hemiplegia-Epilepsy initially involves an infantile presentation of febrile focal motor status epilepticus, with subsequent hemiplegia of the initially affected side. Months to years later, affected children go on to develop a chronic epilepsy with recurrent focal seizures which are often refractory to treatment. This uncommon paediatric epilepsy syndrome is poorly understood, with only a very small minority of cases associated with an underlying genetic or metabolic abnormality. Read More

Epileptic Disord 2018 Dec 10. Epub 2018 Dec 10.

Department of Pediatrics (Neurology), McMaster University, 1280 Main St., W. Hamilton, Ontario L8S 4K1, Canada.

A male neonate presented with seizures at 18 hours of life, characterized by tonic posturing with eye deviation to the right, apnoea, bradycardia, and oxygen desaturation. Initial structural, metabolic, and infectious work-up was unremarkable. He continued to have seizures refractory to a variety of antiepileptic medications. Read More

J Pediatr 2018 Dec 7. Epub 2018 Dec 7.

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

Objectives: To evaluate the proportion of children presenting to the emergency department (ED) with altered mental status who demonstrate nonconvulsive seizures on reduced-lead electroencephalography (EEG), and to further investigate the characteristics, treatment, and outcomes in these patients compared with patients without nonconvulsive seizures.

Study Design: In this retrospective cohort study, we reviewed the database and medical records of pediatric patients (aged <18 years) in a single ED between May 1, 2016, and April 30, 2018. We first determined the proportion of nonconvulsive seizures among patients with altered mental status (Glasgow Coma Scale <15). Read More

Seizure 2018 Oct 6. Epub 2018 Oct 6.

Departments of Neurology and Pediatrics, The Children's Hospital of Philadelphia and the University of Pennsylvania; 3501 Civic Center Blvd. Philadelphia, PA, 19104.

Purpose: To summarize the use of continuous electroencephalographic monitoring (cEEG) in the diagnosis and management of pediatric convulsive status epilepticus (CSE) and subsequent non-convulsive seizures (NCS) with a focus on available guidelines and infrastructure. In addition, we provide an overview of quantitative EEG (QEEG) for the identification of NCS in critically ill children.

Methods: We performed a review of the medical literature on the use of cEEG and QEEG in pediatric CSE. Read More

Brain Dev 2018 Dec 7. Epub 2018 Dec 7.

Division of Neurology, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Thailand. Electronic address:

Background: Super-refractory status epilepticus (SRSE) is a seizure that continues >24 h after anesthesia, or recurs on the reduction of anesthesia. SRSE is extremely difficult-to-control and associated with poor outcome. To date, optimal therapy and outcome data in children is limited. Read More

Epilepsia 2018 Dec 4. Epub 2018 Dec 4.

School of Epidemiology and Public Health, University of Ottawa, Ottawa, Ontario, Canada.

Objective: To assess the benefits and harms of cannabis-based products for pediatric epilepsy.

Methods: We identified in this living systematic review randomized controlled trials (RCTs) and nonrandomized studies (NRSs) involving children with epilepsy treated with cannabis-based products. We searched MEDLINE, Embase, PsycINFO, Cochrane Library, and gray literature (April 25, 2018). Read More

Seizure 2018 Aug 7. Epub 2018 Aug 7.

Department of Neurology, Division of Pediatric Neurology. University of Washington School of Medicine, Seattle, WA, USA.

Purpose: Rescue medications for status epilepticus (SE) have a relatively high rate of failure. The purpose of this review is to summarize the evidence for the efficacy of novel drugs and early polypharmacotherapy for SE.

Method: Literature review. Read More

Arch Dis Child Fetal Neonatal Ed 2018 Nov 24. Epub 2018 Nov 24.

Irish Centre for Fetal and Neonatal Translational Research (INFANT), Cork, Ireland.

Objective: The aim of this multicentre study was to describe detailed characteristics of electrographic seizures in a cohort of neonates monitored with multichannel continuous electroencephalography (cEEG) in 6 European centres.

Methods: Neonates of at least 36 weeks of gestation who required cEEG monitoring for clinical concerns were eligible, and were enrolled prospectively over 2 years from June 2013. Additional retrospective data were available from two centres for January 2011 to February 2014. Read More

J Neurol Sci 2018 Oct 4;396:150-158. Epub 2018 Oct 4.

Department of Pediatrics, Tokyo Women's Medical University, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 162-8666, Japan. Electronic address:

Background: No dosing regimen has been established for the initial treatment of pediatric status epilepticus with intravenous midazolam. We therefore evaluated the efficacy, safety, and pharmacokinetics of bolus and continuous midazolam infusion.

Methods: This open-label, prospective, multicenter study involved 34 Japanese children with status epilepticus unresponsive to diazepam. Read More

Pediatr Crit Care Med 2018 Nov 16. Epub 2018 Nov 16.

Division of Critical Care, Department of Pediatrics, The Children's Hospital at Montefiore, Bronx, NY.

Objectives: Convulsive status epilepticus is a medical emergency. Prompt treatment has been shown to decrease progression to refractory convulsive status epilepticus. We aimed to reduce time to second-line anti-seizure medication through implementation of a standardized treatment protocol. Read More

Clin Case Rep 2018 Nov 12;6(11):2037-2039. Epub 2018 Sep 12.

Penn State Health Milton S. Hershey Medical Center Hershey Pennsylvania.

This case represents a novel association of a DNM1 gene mutation with status epilepticus and progressive bilateral mesial temporal sclerosis. This could have future implications for treatment in patients with DNM1 mutation and refractory epilepsy as the mesial temporal sclerosis may become bilateral, making the patient a poor surgical candidate. Read More

Pediatr Neonatol 2018 Oct 31. Epub 2018 Oct 31.

Chang Gung Children's Hospital Study Group for Children with Encephalitis/Encephalopathy Related Status Epilepticus and Epilepsy (CHEESE), Taoyuan, Taiwan.

Background: Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is an anti-neuronal antibody-mediated inflammatory brain disease that causes severe psychiatric and neurological deficits in previously healthy patients. The aims of this study were to demonstrate the clinical characteristics of patients diagnosed with anti-NMDA receptor encephalitis and to compare the different treatment strategies among these patients.

Methods: Patients presenting with newly acquired psychiatric and/or neurological deficits were studied retrospectively from 2009 to 2017. Read More

Indian Pediatr 2018 Oct;55(10):893-901

Department of Pediatrics, BL Kapur Superspeciality Hospital, Delhi; India.

Surface electroencephalography (EEG) is a useful electrophysiological investigation for evaluating a paroxysmal event in children. It measures the electro potential difference between two points on the scalp. It is a non-invasive tool that analyzes neuronal maturation and abnormal cortical excitability. Read More

Neurobiol Dis 2018 Nov 10;124:141-151. Epub 2018 Nov 10.

Department of Pediatrics, Division of Neurology and Translational Epilepsy Research Program, University of Colorado School of Medicine, Aurora, CO 80045, USA. Electronic address:

Epilepsy is a brain disorder characterized by a predisposition to suffer epileptic seizures. Acquired epilepsy might be the result of brain insults like head trauma, stroke, brain infection, or status epilepticus (SE) when one of these triggering injuries starts a transformative process known as epileptogenesis. There is some data to suggest that, during epileptogenesis, seizures themselves damage the brain but there is no conclusive evidence to demonstrate that spontaneous recurrent seizures themselves injure the brain. Read More

J Neurosci Res 2018 Nov 12. Epub 2018 Nov 12.

Pediatric Research Institute, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, Chongqing, China.

Despite remarkable advances in epilepsy research, prevention and reversal of cognitive deficits following epilepsy remain a challenge. It was reported that the Rho kinase (ROCK) inhibitor fasudil hydrochloride (FH) could improve cognitive deficits in animal models of Alzheimer's disease (AD). Thus, the aim of the present study was to determine whether FH-mediated inhibition of the effects of ROCK signaling could improve cognitive deficits in male rats (postnatal 21-day old) following status epilepticus (SE) induced by lithium-pilocarpin, the therapeutic window of opportunity and to elucidate the underlying mechanisms. Read More

J Clin Neurosci 2018 Nov 8. Epub 2018 Nov 8.

Division of Pediatric Neurology, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.

Purpose: Explore etiology, clinical course and outcome of infant epilepsy in Bhumibol Adulyadej Hospital.

Method: Retrospective and prospective descriptive analysis of infants 1 month to 1 year diagnosed with epilepsy between January 1, 2012, and April 30, 2018.

Results: Total 57 infants. Read More

Neurology 2018 Nov 9;91(22):e2078-e2088. Epub 2018 Nov 9.

From the Department of Clinical and Experimental Epilepsy (S.Z., Z.M., L.H.-H., S.K., S. Balestrini, S.M.S.) and Division of Neuropathology (Z.M., M.T.), UCL Institute of Neurology, London, UK; Clinic of Neurology (S.Z.), Department of Experimental and Clinical Medicine, Marche Polytechnic University, Ancona, Italy; Department of Pediatric Neurology and Neurological Rehabilitation (C.S., T.H., P.W., G.J.K.) and Neurosurgery Clinic and Clinic for Epilepsy Surgery (M.K.), Schön Klinik Vogtareuth; Department of Pediatrics (C.S., M.S.), Children's Hospital Augsburg, Germany; UCL Great Ormond Street Institute of Child Health (J.R.N., K.V., S.M.V., J.H.C.), London, UK; Paediatric Neurology and Neurogenetics Unit and Laboratories (D.M., R.G.), A. Meyer Children's Hospital, University of Florence, Italy; Chalfont Centre for Epilepsy (Z.M., L.H.-H., S.K., S. Balestrini, S.M.S.), Chalfont-St-Peter, Buckinghamshire, UK; CeGaT-Center for Genomics and Transcriptomics (A.P., S. Biskup), Tübingen, Germany; Neurogenetics Unit (M.L.), Department of Medical Genetics, Hospital de São João, Porto, Portugal; Department of Pediatrics and Adolescent Medicine (J.G.), University Medical Center Göttingen; Hospital for Children and Adolescents (A.M.), University Clinic Leipzig, Germany; Freiburg Medical Laboratory (M.J.), Dubai; The Danish Epilepsy Centre (R.S.M., E.G.), Dianalund; Institute for Regional Health Services (R.S.M., E.G.), University of Southern Denmark, Odense; Department of Clinical Genetics (B.S.K.), Odense University Hospital; Hans Christian Andersen Children's Hospital (L.K.H.), Odense, Denmark; Pediatric Neurology and Muscular Diseases Unit (M.S.V., P.S.), Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, and Maternal and Child Health, University of Genoa "G. Gaslini" Institute, Italy; Division of Neurology (K.L.H.), Children's Hospital of Philadelphia, PA; Department of Neurology (S.D., C.L.S.-H.), Division of Neurogenetics, Kennedy Krieger Institute, Baltimore, MD; Center for Genomic Medicine (N.H.-F.), Tohoku University; Department of Pediatrics (N.H.-F.), Tohoku University School of Medicine, Sendai, Japan; Department of Pediatrics (T.T., R.L.) and Institute of Clinical Medicine (K.O.), University of Tartu; Children's Clinic (T.T., R.L.), Department of Radiology (P.I.), and Department of Clinical Genetics, United Laboratories (K.O.), Tartu University Hospital, Estonia; Ludwig-Maximilians-University Munich (I.K.); Department of Pediatric Neurology (A.H.), Clinic Traunstein; Children's Hospital (M.K.), Dr. Horst Schmidt Klinik, Wiesbaden; Altona Children's Hospital (J.H.), Hamburg; Department of Pediatrics (C. Makowski), Technische Universität München, Germany; Department of Clinical Genetics (S.G.), Royal North Shore Hospital, St Leonards; John Hunter Children's Hospital (G.M.S.), New Lambton Heights, New South Wales, Australia; Department of Neurology (R.T.), University Hospital of Wales; Institute of Psychological Medicine and Clinical Neurosciences (R.H.T.), Cardiff University; Division of Neuroradiology (C. Micallef), National Hospital for Neurology and Neurosurgery, London; Department of Brain Repair & Rehabilitation (D.J.W.), Stroke Research Centre, UCL Institute of Neurology, London, UK; Paracelsus Medical University (G.J.K.), Salzburg, Austria; and IRCCS Stella Maris Foundation (R.G.), Pisa, Italy.

Objective: To characterize the neurologic phenotypes associated with mutations and to seek genotype-phenotype correlation.

Methods: We analyzed clinical, EEG, and neuroimaging data of 44 new and 55 previously reported patients with mutations.

Results: Childhood-onset focal seizures, frequently complicated by status epilepticus and resistance to antiepileptic drugs, was the most common phenotype. Read More

Seizure 2018 Sep 18. Epub 2018 Sep 18.

Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA. Electronic address:

Purpose: Status epilepticus is an often apparently randomly occurring, life-threatening medical emergency which affects the quality of life in patients with epilepsy and their families. The purpose of this review is to summarize information on ambulatory seizure detection, seizure prediction, and status epilepticus prevention.

Method: Narrative review. Read More

Child Adolesc Psychiatr Clin N Am 2019 Jan 11;28(1):21-32. Epub 2018 Sep 11.

Department of Anesthesiology and Perioperative Medicine, Nemours/Alfred I. DuPont Hospital for Children, 1600 Rockland Road, Wilmington, DE 19803, USA.

Proper planning and communication between psychiatry and anesthesiology teams is vital to conferring the greatest therapeutic benefit to children presenting for electroconvulsive therapy while minimizing risk. Anesthesia for the child undergoing electroconvulsive therapy should ideally provide deep hypnosis, ensure muscle relaxation to reduce injury, have minimal effect on seizure dynamics, and allow for rapid recovery to baseline neurologic and cardiopulmonary status. Unique factors for pediatric electroconvulsive therapy include the potential need for preoperative anxiolytic and inhalational induction of anesthesia, which must be weighed against the detrimental effects of anesthetic agents on the evoked seizure quality required for a successful treatment. Read More

J Child Neurol 2018 Oct 26:883073818811538. Epub 2018 Oct 26.

2 Division of Pediatric Neurology, Department of Pediatrics, Istanbul Medical Faculty, Istanbul, Turkey.

Dravet syndrome is a rare and progressive epileptic encephalopathy of infancy. Stiripentol reduces the seizure frequency in patients with Dravet syndrome. We evaluated the clinical characteristics of patients with Dravet syndrome and their response to stiripentol. Read More

Pediatr Neurol 2018 Nov 10;88:59-64. Epub 2018 Aug 10.

Wayne State University School of Medicine, Carman and Ann Adams Department of Pediatrics, Division of Emergency Medicine, Children's Hospital of Michigan, Detroit, Michigan. Electronic address:

Background: Seizures are a common presenting symptom to the emergency department (ED) in children with intraventricular shunts (IVS). The incidence of shunt malfunction and the yield of imaging studies in children with IVS presenting with seizures is unknown.

Objectives: We assessed the utility and diagnostic yield of evaluation for shunt malfunction in patients with IVS with seizure and identified clinical predictors for shunt malfunction in these children. Read More

Pediatr Neurol 2018 Dec 4;89:26-30. Epub 2018 Aug 4.

Helen DeVos Children's Hospital, Grand Rapids, Michigan; College of Human Medicine Department of Pediatrics and Human Development, Michigan State University, Grand Rapids, Michigan.

Background: Infantile epileptic encephalopathy is a heterogeneous condition that has been associated with variants in more than 200 genes. The variability in findings and prognosis creates challenges to making the correct diagnosis and initiating the appropriate therapy. Biallelic variants in NARS2, a mitochondrial aminoacyl-tRNA synthetase gene, were recently associated with neurodegenerative disorders that include epilepsy. Read More

Pediatr Neurol 2018 Dec 4;89:31-38. Epub 2018 Aug 4.

Oregon Health and Science University, Department of Pediatrics, Division of Pediatric Critical Care, Portland, Oregon.

Background: Disorders requiring pediatric neurocritical care (PNCC) affect thousands of children annually. We aimed to quantify the burden of PNCC through generation of national estimates of disease incidence, utilization of critical care interventions (CCI), and hospital outcomes.

Methods: We performed a retrospective cohort analysis of the Kids Inpatient Database over three years to evaluate pediatric traumatic brain injury, neuro-infection or inflammatory diseases, status epilepticus, stroke, hypoxic ischemic injury after cardiac arrest, and spinal cord injury. Read More

Brain Dev 2018 Oct 13. Epub 2018 Oct 13.

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

Background: Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is characterized by biphasic seizures and impaired consciousness. Takotsubo cardiomyopathy (TTC), which is typically triggered by psychological or physical stress, is characterized by transient myocardial dysfunction affecting the left ventricular apex. Recent reports have suggested that seizures can also trigger TTC. Read More

Neurophysiol Clin 2018 Oct 12. Epub 2018 Oct 12.

Division of pulmonary and critical care, department of pediatrics, faculty of medicine, Chulalongkorn University, Thailand.

Objective: To determine the prognostic value of continuous electroencephalography (EEG) in children undergoing therapeutic hypothermia after cardiac arrest.

Method: We retrospectively reviewed medical records and continuous EEG of all patients undergoing therapeutic hypothermia after cardiac arrest from November 2013 to September 2016. Demographic, clinical data and immediate complications were collected. Read More

Eur J Paediatr Neurol 2018 Sep 28. Epub 2018 Sep 28.

Sydney Children's Hospital Network (SCHN), Australia; The University of New South Wales, Australia.

Purpose: Neurological complications of influenza cause significant disease in children. Central nervous system inflammation, the presumed mechanism of influenza-associated encephalopathy, is difficult to detect. Characteristics of children presenting with severe neurological complications of influenza, and potential biomarkers of influenza-associated encephalopathy are described. Read More

Epilepsia 2018 Nov 8;59(11):e166-e171. Epub 2018 Oct 8.

Department of Pharmacology, Medical University of Innsbruck, Innsbruck, Austria.

There exists solid evidence that endogenous galanin and galanin agonists exert anticonvulsive actions mediated both by galanin 1 receptor (GAL1-R) and galanin 2 receptor (GAL2-R). We have now investigated whether depletion of the recently identified third galanin receptor, GAL3-R, and that of GAL2-R, alters the threshold to the systemically applied γ-aminobutyric acid (GABA) antagonist pentylenetetrazole (PTZ) or to intrahippocampally administered kainic acid (KA). In neither model, GAL3-KO mice differed in their latency to the first seizure, mean seizure duration, total number of seizures, or time spent in seizures compared to wild-type controls. Read More

Cell Physiol Biochem 2018 2;50(1):150-168. Epub 2018 Oct 2.

Department of Neurology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan, Taiwan.

Background/aims: Immunological mechanisms can be triggered as a response to central nervous system insults and can lead to seizures. In this study an investigation was made to determine if glatiramer acetate (GA), an immunomodulator currently used in the treatment of multiple sclerosis, could protect rats from pilocarpine-induced seizures and chronic epilepsy.

Methods: Two groups of adult male Sprague-Dawley rats, experimental (GA) and control, were used in the study. Read More

J Neurosci Rural Pract 2018 Oct-Dec;9(4):487-491

Department of Psychiatry, Government Medical College, Srinagar, Jammu and Kashmir, India.

Background And Objectives: Despite advance in treatment of status epilepticus (SE), a major neurological emergency, it is still associated with mortality and morbidity. The objective of our study was to estimate sociodemographic profile, semiology, and etiology in the children with SE admitted in pediatric intensive care.

Materials And Methods: Children between 1 month and 18 years with continuous seizure activity of >5 min or two or more sequential seizures without full recovery of consciousness between seizures, admitted in the Pediatric Intensive Care Unit of the Department of Pediatrics, Government Medical College, Srinagar, were included in the study. Read More

Seizure 2018 Aug 29. Epub 2018 Aug 29.

Department of Pediatrics, Weill Cornell Medicine, New York, NY, United States; New-York Presbyterian Hospital, New York, NY, United States; Department of Healthcare Policy & Research, Weill Cornell Medicine, New York, NY, United States. Electronic address:

Purpose: To summarize the epidemiology, morbidity, mortality, and costs of status epilepticus (SE) in the pediatric population.

Method: Review of the medical literature.

Results: The overall incidence of pediatric SE is roughly 20 per 100,000 children per year, with overall mortality of 3%. Read More

CPT Pharmacometrics Syst Pharmacol 2018 Nov 28;7(11):718-727. Epub 2018 Sep 28.

Division of Pharmacotherapy and Experimental Therapeutics, UNC Eshelman School of Pharmacy, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA.

Diazepam is labeled for status epilepticus (SE) in children, but there are limited data characterizing its disposition in pediatric patients. We developed a population pharmacokinetic (PK) model of i.v. Read More

Epilepsia 2018 Nov 6;59(11):2005-2018. Epub 2018 Sep 6.

Department of Anatomy/Neurobiology, University of California, Irvine, Irvine, California.

Objective: A subset of children with febrile status epilepticus (FSE) are at risk for development of temporal lobe epilepsy later in life. We sought a noninvasive predictive marker of those at risk that can be identified soon after FSE, within a clinically realistic timeframe.

Methods: Longitudinal T -weighted magnetic resonance imaging (T WI MRI) of rat pups at several time points after experimental FSE (eFSE) was performed on a high-field scanner followed by long-term continuous electroencephalography. Read More

Pediatr Crit Care Med 2018 Dec;19(12):1162-1167

Department of Neurology, New York - Presbyterian, Columbia University Herbert and Florence Irving Medical Center, New York, NY.

Objectives: Standards for neuromonitoring during extracorporeal membrane oxygenation support do not currently exist, and there is wide variability in practice. We present our institutional experience at an academic children's hospital since establishment of a continuous electroencephalography monitoring protocol for extracorporeal membrane oxygenation patients.

Design: Retrospective, single-center study. Read More

Seizure 2018 Sep 14. Epub 2018 Sep 14.

Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Boston, MA, USA.

Purpose: To summarize the evidence regarding dietary, immunological, surgical, and other emerging treatments for refractory status epilepticus (RSE)/super-RSE (SRSE).

Methods: Narrative literature review including relevant human studies.

Results: Hypothermia and brenaxolone were tested in randomized controlled trials for RSE/SRSE management, while other interventions have only limited evidence for their efficacy and safety. Read More

Zhonghua Bing Li Xue Za Zhi 2018 Sep;47(9):676-681

Department of Pathology, Beijing Haidian Hospital/Haidian Section of Peking University Third Hospital, Beijing 100080, China.

To investigate the clinicopathologic features of Rasmussen syndrome (RS) and to raise awareness of this rare disease. Clinicopathologic data of 4 cases of RS were retrospectively analyzed at Beijing Haidian Hospital from 2008 to 2016. The clinical manifestations included epilepsia partialis continua and progressive neurologic deficits in all patients. Read More

Medicina (B Aires) 2018 ;78 Suppl 2:12-17

Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina. E-mail:

Status epilepticus (SE) is one of the most common neurologic emergencies in pediatrics. It is a condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which leads to abnormally, prolonged seizures. This definition provides a good guidance, when emergency treatment must be considered. Read More

Anaesth Intensive Care 2018 Sep;46(5):516-528

Professor, Departments of Medicine, Anesthesiology, Neurology and Neurosurgery, NYU School of Medicine, New York, NY, USA.

Our goal was to provide comprehensive data on the effectiveness of ketamine in refractory status epilepticus (RSE) and to describe the potential consequences of long-term ketamine infusion. Ketamine, an N-methyl D-aspartate (NMDA) receptor antagonist, blocks excitatory pathways contributing to ongoing seizure. While ketamine use is standard in anaesthetic induction, no definitive protocol exists for its use in RSE, and little is known about its adverse effects in long-term, high-dose administration. Read More

Zhonghua Er Ke Za Zhi 2018 Sep;56(9):667-673

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

To summarize the clinical features of TBC1D24 gene mutations associated with epilepsy. All the patients with TBC1D24 gene compound heterozygous mutations were retrospectively collected at the Pediatric Department of Peking University First Hospital from March 2015 to July 2017, and the features of clinical manifestations, electroencephalogram, and neuroimaging were analyzed. Eighteen cases with TBC1D24 gene compound heterozygous mutations were included. Read More

Neurology 2018 Sep 31;91(12):e1112-e1124. Epub 2018 Aug 31.

From the Department of Clinical Neurophysiology (E.G., S.B.), Danish Epilepsy Centre, Dianalund; Institute for Regional Health Services (E.G., K.M.J., R.S.M.), University of Southern Denmark, Odense, Denmark; Neuroscience Department (C.M., R.G., M.M.), Children's Hospital A. Meyer, University of Florence; Department of Neuroscience (M.T., N.S., F.V.), Bambino Gesù Children's Hospital, IRCCS, Rome, Italy; Division of Neurology (M.P.F., I.H.), The Children's Hospital of Philadelphia; Departments of Pediatrics and Neurology (M.P.F., I.H.), Perelman School of Medicine at the University of Pennsylvania, Philadelphia; Universitätskinderklinik Tübingen (M.A., M.W.), Germany; Department of Neurology (K.H.), Royal Children's Hospital Melbourne; Department of Paediatrics (K.H.), University of Melbourne; Australia Neurosciences Group (K.H.), Murdoch Children's Research Institute, Melbourne, Australia; Servizio di Neuropsichiatria Infantile (F.D., E.F.), Policlinico G.B. Rossi, Universita Degli Studi di Verona; Department of Child Neurology (S.S., G.A.), Ospedale Pediatrico G. Salesi-Ospedali Riuniti, Ancona, Italy; Division of Clinical Neurophysiology (B.B.), Children's Research Center, University Children's Hospital Zurich, Switzerland; Brain and Behaviour Department (S.M.), University of Pavia; Department of Pediatric Neuroradiology (A.P.), IRCCS "C. Mondino" National Neurological Institute, Pavia, Italy; Department of Epilepsy Genetics (K.J., R.S.M.), Danish Epilepsy Centre Dianalund; Department of Child Neurology (B.J.), Danish Epilepsy Centre, Dianalund, Denmark; Cytogenetic and Molecular Genetic Laboratory (S.R., F.C.), Istituto Auxologico Italiano, IRCCS, Milano, Italy; Department of Adult Neurology (G.R.), Danish Epilepsy Centre, Dianalund; University of Copenhagen (G.R.), Denmark; Struttura Complessa di Neurologia Pediatrica Ospedale Vittore Buzzi (P.V.), Milano; Dipartimento di Scienze Biomediche e Cliniche L. Sacco (P.V.), Università di Milano, Italy; Århus University (S.B.), Denmark; Department of Child Neurology (I.E.S.), University of Melbourne, Austin Health, Florey Institute; and Department of Child Neurology (I.E.S.), The Royal Children's Hospital, Melbourne, Australia.

Objective: To delineate the electroclinical features of infantile developmental and epileptic encephalopathy (EIEE13, OMIM #614558).

Methods: Twenty-two patients, aged 19 months to 22 years, underwent electroclinical assessment.

Results: Sixteen of 22 patients had mildly delayed development since birth. Read More

Seizure 2018 Aug 6. Epub 2018 Aug 6.

Neurosciences Unit, University College London Great Ormond Street Institute of Child Health, London, UK; Department of Neurological Sciences, University of Vermont, Burlington, VT, United States. Electronic address:

Purpose: To summarize the pathophysiology of convulsive status epilepticus (SE) with a focus on practical implications for treatment.

Method: Narrative review of the medical literature on the pathophysiology of convulsive SE. We considered both animal models of SE and clinical studies. Read More

Lancet Child Adolesc Health 2018 Feb 7;2(2):81-83. Epub 2017 Dec 7.

Department of Neurology and Department of Pediatrics, Children's Hospital of Philadelphia, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

Int J Anal Chem 2018 1;2018:2583215. Epub 2018 Aug 1.

Department of Biochemistry, Kasturba Medical College, Manipal Academy of Higher Education, Manipal 576104, India.

Pyridoxine dependent epilepsy is a condition where the affected infant or child has prolonged seizures (status epilepticus), which are nonresponsive to anticonvulsant therapy but can be treated with pharmacological doses of pyridoxine. If identified earlier and treated prophylactically with pyridoxine, severe brain damage due to seizures can be prevented. Alpha-amino adipic semialdehyde (AASA), piperidine-6-carboxylic acid (P6C), and pipecolic acid (PA) are known biomarkers of pyridoxine dependent epilepsy. Read More

Brain Dev 2018 Nov 23;40(10):884-890. Epub 2018 Aug 23.

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

Background: Fosphenytoin (fPHT) and continuous intravenous midazolam (cMDL) had commonly been used as second-line treatments for pediatric status epilepticus (SE) in Japan. However, there is no comparative study of these two treatments.

Methods: We included consecutive children who 1) were admitted to Kobe Children's Hospital because of convulsion with fever and 2) were treated with either fPHT or cMDL as second-line treatment for convulsive SE lasting for longer than 30 min. Read More

Pediatr Nephrol 2018 Nov 23;33(11):2201-2204. Epub 2018 Aug 23.

Department of Pediatrics, Schulich School of Medicine and Dentistry, University of Western Ontario, Children's Hospital, London Health Science Centre, 800 Commissioners Road East, London, ON, N6A 5W9, Canada.

Background: Galloway-Mowat syndrome (GAMOS) (OMIM #251300) is a severe autosomal recessive disease characterized by the combination of early-onset steroid-resistant nephrotic syndrome (SRNS) and microcephaly with brain anomalies caused by WDR73 as well as OSGEP, TP53RK, TPRKB, or LAGE3 mutations.

Objective: We report on the hitherto undescribed urological and nephrological complications of the homozygous c.974G>A (p. Read More

Pediatr Neurol 2018 Oct 4;87:65-69. Epub 2018 Jul 4.

Neurology Divison, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas.

Objectives: We describe a 13-year-old girl with a past medical history of epilepsy, intellectual impairment, dysphagia with gastric tube dependence, and autism spectrum disorder who presented with focal status epilepticus.

Methods: Video-electroencephalography revealed left occipital pseudoperiodic epileptiform discharges and frequent seizures originating from the left hemisphere. The seizure was refractory to antiepileptic medications and pharmacologic coma. Read More

Epilepsia 2018 Sep 22;59(9):1705-1717. Epub 2018 Aug 22.

Reference Center for Rare Epilepsies, APHP, Necker-Enfants Malades Hospital, Paris, France.

Objective: To evaluate continuing stiripentol treatment in adulthood in Dravet syndrome (DS).

Method: Longitudinal data were collected from the last visit prior to age 15 years (V ) to the last visit in adulthood (V ) in the 40 DS patients (32 typical, eight atypical) of a French historical cohort (Paris) of subjects who continued stiripentol from childhood or adolescence to adulthood.

Results: At V (18-40 years, median = 23 years), all the patients were still receiving stiripentol (exposure = 3-24 years, median = 18 years), associated with clobazam (40/40), valproate (39/40), and topiramate (21/40). Read More

Pediatrics 2018 Sep 8;142(3). Epub 2018 Aug 8.

Epilepsy Center, Ann and Robert H. Lurie Children's Hospital of Chicago and Department of Pediatrics, Feinberg School of Medicine, Northwestern University, Chicago, Illinois

Objectives: We assessed the adherence to neuroimaging guidelines and the diagnostically relevant yield of neuroimaging in newly presenting early life epilepsy (ELE).

Methods: There were 775 children with a new diagnosis of epilepsy (<3 years old at onset) who were recruited through the ELE study at 17 US pediatric epilepsy centers (2012-2015) and managed prospectively for 1 year. The data were analyzed to assess the proportion of children who underwent neuroimaging, the type of neuroimaging, and abnormalities. Read More

Pediatr Neurol 2018 Sep 11;86:33-41. Epub 2018 Jul 11.

Departments of Neurology and Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts. Electronic address:

Objective: We aimed to evaluate and compare the status epilepticus treatment pathways used by pediatric status epilepticus research group (pSERG) hospitals in the United States and the American Epilepsy Society (AES) status epilepticus guideline.

Methods: We undertook a descriptive analysis of recommended timing, dosing, and medication choices in 10 pSERG hospitals' status epilepticus treatment pathways.

Results: One pathway matched the timeline in the AES guideline; nine pathways described more rapid timings. Read More

Epilepsy Res 2018 Oct 20;146:28-35. Epub 2018 Jul 20.

Department of Neurology, Children's Hospital of Chongqing Medical University, China; Ministry of Education Key Laboratory of Child Development and Disorders, China; Key Laboratory of Pediatrics in Chongqing, CSTC2009CA5002, China; Chongqing International Science and Technology Cooperation Center for Child Development and Disorders, China. Electronic address:

Objective: To assess the dynamic changes in the average and peak spectral power of fast ripples (FRs) in the hippocampi after interventions with valproate sodium (VPA), carbenoxolone (CBX) and quinine (QUIN).

Method: Adult rats were used to establish a lithium-pilocarpine (pilo) epileptic model, and were assigned to a lithium-pilocarpine (PILO), VPA + PILO, QUIN + PILO or CBX + PILO group. Intracranial electroencephalography was performed before and after status epilepticus (SE). Read More