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Child Neurol Open 2019 5;6:2329048X19826288. Epub 2019 Feb 5.

Department of Radiology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea.

Acute encephalopathy with biphasic seizures and late reduced diffusion is a subtype of acute encephalopathy described in a cohort of Japanese children. Few cases have been reported in countries other than Japan. It is characterized clinically by biphasic seizures and late reduced subcortical diffusion on magnetic resonance imaging (MRI). Read More

Epilepsy Behav 2019 Feb 16;93:7-11. Epub 2019 Feb 16.

Department of Neurology, University of Arkansas for Medical Sciences, Little Rock, AR, United States of America; Minnesota Epilepsy Group, P.A., St. Paul, MN, United States of America.

Purpose: The prevalence and characteristics of seizure and epilepsy research published in nonneurology journals are unknown. Characterizing this published research allows for insight into the relevance of seizures and epilepsy in other specialties and may increase opportunity for cross-specialty collaboration.

Methods: In this observational study, we reviewed the top five highly cited clinical journals within eleven specialties in the InCites Journal Citation Reports (JCR) database (2016). Read More

Ann Neurol 2019 Feb 18. Epub 2019 Feb 18.

Department of Neurology, Mayo Clinic, Rochester, MN, USA, 55905.

Objectives: We recently reported successful treatment of a child with febrile infection-related epilepsy syndrome (FIRES), a subtype of new-onset refractory status epilepticus (NORSE), with the recombinant interleukin-1 (IL1) receptor antagonist (IL1RA) anakinra. On this basis, we tested whether endogenous IL1RA production or function is deficient in FIRES patients.

Methods: Levels of IL1β and IL1RA were measured in serum and CSF. Read More

Curr Treat Options Neurol 2019 Feb 18;21(2). Epub 2019 Feb 18.

Division of Neurology, Department of Pediatrics, Nationwide Children's Hospital, Ohio State University, 611 E Livingston Avenue FB4, Columbus, OH, 43205, USA.

Purpose Of Review: The purpose of this review is to report recent advances in treatment of neonatal seizures, with a specific focus on new literature since a 2013 systematic review performed by this author (Slaughter) and others. There is a paucity of data with regard to well-defined status epilepticus (SE) in neonates, so treatment of recurrent seizures was also included in this inquiry. We aimed to summarize the efficacy and safety profiles of current therapeutic options as well as describe trends in medication selection in the neonatal intensive care unit (NICU) setting. Read More

Epileptic Disord 2019 Feb 15. Epub 2019 Feb 15.

Department of Neurological Sciences, Larner College of Medicine, University of Vermont, Burlington, Vermont, USA.

Electrical status epilepticus in sleep (ESES) is an age-related, self-limited epileptic encephalopathy. The syndrome is characterized by cognitive and behavioral abnormalities and a specific EEG pattern of continuous spikes and waves during slow-wave sleep. While spikes and sharp waves are known to result in transient cognitive impairment during learning and memory tasks performed during the waking state, the effect of epileptiform discharges during sleep on cognition and behavior is unclear. Read More

J Clin Neurophysiol 2019 Jan 24. Epub 2019 Jan 24.

Division of Neurology, Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Detroit, Michigan, U.S.A.

Purpose: The Critical Care Continuous EEG Task Force of the American Clinical Neurophysiology Society recommends continuous EEG (cEEG) monitoring in patients with persistent encephalopathy following convulsive status epilepticus. This recommendation is based on data, which correlates prolonged nonconvulsive seizures and nonconvulsive status epilepticus with worse neurologic outcomes. Compliance with these recommendations may be limited by barriers such as inadequate resource and staff availability. Read More

Childs Nerv Syst 2019 Mar 23;35(3):463-468. Epub 2019 Jan 23.

Department of Biostatistics, Cukurova University, Adana, Turkey.

Purpose: We aimed to determine the characteristics of epileptic seizures that significantly affect the cognitive functions of 83 patients followed with tuberous sclerosis complex (TSC), their resistance to treatment and risk factors causing this resistance.

Materials-methods: In order to determine the prognosis, the seizure-free/seizure-controlled group and the group with refractory seizures were compared. In addition, risk factors affecting cognitive functions in the patients were determined. Read More

Brain 2019 Jan 21. Epub 2019 Jan 21.

British Columbia Children's Hospital Research Institute, Vancouver, BC, Canada.

Biallelic pathogenic variants in PLPBP (formerly called PROSC) have recently been shown to cause a novel form of vitamin B6-dependent epilepsy, the pathophysiological basis of which is poorly understood. When left untreated, the disease can progress to status epilepticus and death in infancy. Here we present 12 previously undescribed patients and six novel pathogenic variants in PLPBP. Read More

Indian J Crit Care Med 2018 Dec;22(12):875-878

Department of Pediatrics, Kalinga Institute of Medical Sciences, Bhubaneswar, Odisha, India.

Objective: The main objective is to assess the challenges in diagnosis and treatment while managing seronegative cases of autoimmune encephalitis (AIE) in Indian children.

Methods: A cohort study of patients with AIE was done where clinical presentations, investigations, management were analyzed and these patients were followed up to assess the evolution of the disease.

Results: Nine patients were included in the study. Read More

Epilepsy Res 2019 Feb 14;150:66-69. Epub 2019 Jan 14.

Research Institute of the McGill University Health Center, 2155 Guy Street, Montreal, PQ, H3H 2L9, Canada; Department of Pediatrics, Division of Child Neurology, Montreal Children's Hospital, McGill University Health Centre, 1001 Boulevard Décarie, Montreal, PQ, H4A 3J1, Canada.

We aimed to describe families with genetic epilepsy with febrile seizures plus (GEFS+) in which individuals suffered sudden unexpected death. The Epilepsy Pharmacogenomics Research Database was reviewed for GEFS + families in which at least one individual had suffered sudden death, and two families were identified. In Family A, five males had febrile seizures and one girl had febrile seizures plus. Read More

Brain Dev 2019 Jan 9. Epub 2019 Jan 9.

Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University, School of Medicine, Seoul, Republic of Korea. Electronic address:

Objectives: Super-refractory status epilepticus (SRSE) is one of the most challenging issues in intensive care units (ICUs) in that it is associated with high morbidity and mortality. Although the ketogenic diet (KD) has been reported to be effective in treating of SRSE, the use of the diet as therapy can be complicated by concomitant medical problems specific to critically ill patients. In this study, we aimed to describe our experience of the KD for SRSE patients in ICUs. Read More

Mol Neurobiol 2019 Jan 8. Epub 2019 Jan 8.

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

The peroxisome proliferator-activated receptor (PPAR) family, type II nucleus receptors have been successfully tested for their neuroprotective potential in certain central nervous system diseases. The aim of the present study was to determine if modulation by PPAR-γ could attenuate pilocarpine-induced seizures and decrease neuronal excitability. Adult male C57BL/6 mice were divided into two groups: one group received pretreatment with pioglitazone and the other received dimethyl sulfoxide (DMSO) for a period of 2 weeks. Read More

J Clin Med 2019 Jan 2;8(1). Epub 2019 Jan 2.

Department of Anesthesiology, AOU Policlinico-Vittorio Emanuele, University of Catania Via S. Sofia, 78, 95123 Catania, Italy.

Seizures are defined as a transient occurrence of signs and symptoms due to the abnormal, excessive, or synchronous neuronal activity in the brain characterized by abrupt and involuntary skeletal muscle activity. An early diagnosis, treatment, and specific medical support must be performed to prevent Status Epilepticus (SE). Seizure onset, especially in the child population, is related to specific risk factors like positive family history, fever, infections, neurological comorbidity, premature birth, mother's alcohol abuse, and smoking in pregnancy. Read More

Epilepsy Behav Case Rep 2018 17;10:141-144. Epub 2018 Jul 17.

Department of Pediatrics (Division of Neurology), David Geffen School of Medicine and UCLA Mattel Children's Hospital, Los Angeles, CA, USA.

We present the case of a child with long-standing, super-refractory status epilepticus (SRSE) who manifested prompt and complete resolution of SRSE upon exposure to pure cannabidiol. SRSE emerged in the context of remote suspected encephalitis with previously well-controlled epilepsy. We discuss the extent to which response may be specifically attributed to cannabidiol, with consideration and discussion of multiple potential drug-drug interactions. Read More

Curr Neuropharmacol 2018 Dec 27. Epub 2018 Dec 27.

Neurology Clinic, University of Perugia - S. Maria della Misericordia Hospital, Perugia. Italy.

After more than a century from its discovery, valproic acid (VPA) still represents one of the most efficient antiepileptic drugs (AEDs). Pre and post-synaptic effects of VPA depend on a very broad spectrum of actions, including the regulation of ionic currents and the facilitation of GABAergic over glutamatergic transmission. As a result, VPA indirectly modulates neurotransmitter release and strengthens the threshold for seizure activity. Read More

JAMA Neurol 2018 Dec 21. Epub 2018 Dec 21.

Institute of Human Genetics, University Medical Center Leipzig, Leipzig, Germany.

Importance: The identification and understanding of the monogenic causes of neurodevelopmental disorders are of high importance for personalized treatment and genetic counseling.

Objective: To identify and characterize novel genes for a specific neurodevelopmental disorder characterized by refractory seizures, respiratory failure, brain abnormalities, and death in the neonatal period; describe the outcome of glutaminase deficiency in humans; and understand the underlying pathological mechanisms.

Design, Setting, And Participants: We performed exome sequencing of cases of neurodevelopmental disorders without a clear genetic diagnosis, followed by genetic and bioinformatic evaluation of candidate variants and genes. Read More

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;20(6):545-550

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;20(6):541-544

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 2019 Jan 4;60(1):6-19. 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 2019 Jan 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 2019 Jan;20(1):47-53

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

Pediatr Clin North Am 2018 12;65(6):1119-1134

Pediatric Critical Care Medicine, University of Michigan School of Medicine, 1500 East Medical Center Drive, SPC 5243, Ann Arbor, MI 48109-5243, USA. Electronic address:

In caring for critically ill children, recognition and management often begins in the pediatric emergency department. A seamless transition in care is needed to ensure appropriate care to the sickest of children. This review covers the management of critically ill children in the pediatric emergency department beyond the initial stabilization for conditions such as acute respiratory failure and pediatric acute respiratory distress syndrome, traumatic brain injury, status epilepticus, congenital heart disease, and metabolic emergencies. 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 2019 Apr 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 2019 Apr 12;97(4):506-519. 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 2019 Jan 8;59:119-123. 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 2019 Feb 12;49(1):41-47. 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 2019 Jan 28;23(1):204-213. 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