1,183 results match your criteria Pediatrics Status Epilepticus


Quality of life and correlating factors in children, adolescents with epilepsy, and their caregivers: A cross-sectional multicenter study from Germany.

Seizure 2019 Mar 29;69:92-98. Epub 2019 Mar 29.

Epilepsy Center Hessen and Department of Neurology, Philipps-University Marburg, Marburg (Lahn), Germany; Epilepsy Center Frankfurt Rhine-Main and Department of Neurology, Goethe University Frankfurt, Frankfurt am Main, Germany; LOEWE Center for Personalized Translational Epilepsy Research (CePTER), Goethe-University Frankfurt, Frankfurt am Main, Germany. Electronic address:

Purpose: To identify factors correlating with poorer quality of life (QoL) in children and adolescents with epilepsy and regarding QoL and depression of their caregivers in Germany.

Method: A cross-sectional multicenter study on QoL and depression was performed in two representative German states (Hessen and Schleswig-Holstein). Variance analysis, linear regression, and bivariate correlation were used to identify correlating factors for poorer QoL and symptoms of depression. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10591311183052
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http://dx.doi.org/10.1016/j.seizure.2019.03.016DOI Listing
March 2019
1 Read

A school-aged boy with super-refractory status epilepticus secondary to cortical dysplasia treated with dexmedetomidine.

J Clin Neurosci 2019 Apr 3. Epub 2019 Apr 3.

University of Florida, Department of Pediatrics, Division of Neurology, 1600 SW Archer Road, Gainesville, FL 32608, United States. Electronic address:

This case report details a novel approach to the management of super-refractory status epilepticus (SRSE) in a boy with new-onset seizures progressing to SRSE. After exhausting multiple medications, dexmedetomidine, an α2-adrenoreceptor agonist used for sedation, stopped his SRSE. Dexmedetomidine provides a unique mechanism of action to treat this condition. Read More

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http://dx.doi.org/10.1016/j.jocn.2019.03.050DOI Listing
April 2019
1 Read

Mouse model of human poisonings with tetramethylenedisulfotetramine: Characterization of the effect of exposure route on syndrome outcomes.

Toxicol Lett 2019 Jun 30;308:50-55. Epub 2019 Mar 30.

Department of Public Health, Division of Environmental Health Science, School of Health Sciences and Practice, New York Medical College, 40 Sunshine Cottage Rd, Valhalla, New York, 10595, USA; Department of Cell Biology and Anatomy, New York Medical College, 40 Sunshine Cottage Rd, Valhalla, New York, 10595, USA; Department of Medicine, Division of Pulmonary and Critical Care Medicine, Rutgers-Robert Wood Johnson Medical School, Piscataway, 675 Hoes Ln W, New Jersey, 08854, USA.

Tetramethylenedisulfotetramine (TMDT) is a synthetic neurotoxic rodenticide and potential chemical threat agent. Signs of TMDT poisoning include convulsions which can progress into status epilepticus and death. Although clinical reports clearly show that poisoning via food and drink is the main route of exposure, experimental studies have primarily utilized parenteral routes. Read More

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http://dx.doi.org/10.1016/j.toxlet.2019.03.014DOI Listing
June 2019
3 Reads

A De Novo Dominant Negative Mutation in DNM1L Causes Sudden Onset Status Epilepticus with Subsequent Epileptic Encephalopathy.

Neuropediatrics 2019 Apr 2. Epub 2019 Apr 2.

Department of Pediatrics, Kinderklinik Muenchen Schwabing, Klinikum Schwabing, StKM GmbH und Klinikum rechts der Isar, Technical University of Munich, Munich, Germany.

Mitochondrial dynamics such as fission and fusion play a vital role in normal brain development and neuronal activity. encodes a dynamin-related protein 1 (Drp1), which is a GTPase essential for proper mitochondrial fission. The clinical phenotype of mutations depends on the degree of mitochondrial fission deficiency, ranging from severe encephalopathy and death shortly after birth to initially normal development and then sudden onset of refractory status epilepticus with very poor neurologic outcome. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0039-1685217
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http://dx.doi.org/10.1055/s-0039-1685217DOI Listing
April 2019
5 Reads

Status epilepticus associated with Mycoplasma pneumoniae encephalitis in children: good prognosis following early diagnosis and treatment.

Chin Med J (Engl) 2019 Mar 27. Epub 2019 Mar 27.

Department of Neurology, Children's Hospital Affiliated to Capital Institute of Pediatrics, Beijing 100020, China.

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http://dx.doi.org/10.1097/CM9.0000000000000233DOI Listing
March 2019
1 Read

Focal Status Epilepticus With Unilateral Brain Edema: An Expanding Spectrum.

Pediatr Neurol 2019 Feb 8. Epub 2019 Feb 8.

Pediatric Neurology Division, Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Electronic address:

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.02.002DOI Listing
February 2019
1 Read

Paroxysmal non-epileptic events in the pediatric emergency department.

Eur Rev Med Pharmacol Sci 2019 Mar;23(5):2188-2193

Department of Pediatrics, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.

Paroxysmal non-epileptic events (PNEs), or pseudoseizures (PS) resemble epileptic seizures. They are considered part of a personality disorder and have a higher incidence among adolescents. Patients describe episodes (lasting up to 20 minutes) of loss of consciousness, twitching or jerking and unusual emotional states. Read More

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http://dx.doi.org/10.26355/eurrev_201903_17265DOI Listing
March 2019
1 Read

Anticonvulsant and Neuroprotective Effects of Dexmedetomidine on Pilocarpine-Induced Status Epilepticus in Rats Using a Metabolomics Approach.

Med Sci Monit 2019 Mar 20;25:2066-2078. Epub 2019 Mar 20.

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 (mainland).

BACKGROUND Status epilepticus (SE) is the most extreme form of seizure. It is a medical and neurological emergency that requires prompt and appropriate treatment and early neuroprotection. Dexmedetomidine (DEX) is mainly used for its sedative, analgesic, anxiolytic, and neuroprotective effects with light respiratory depression. Read More

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http://dx.doi.org/10.12659/MSM.912283DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6437718PMC
March 2019
1 Read

A newborn with seizures born to a mother diagnosed with primary carnitine deficiency.

BMC Pediatr 2019 Mar 18;19(1):79. Epub 2019 Mar 18.

Department of Neonatology, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Zhejiang, 325027, Wenzhou, China.

Background: Maternofetal carnitine transport through the placenta is the main route of fetal carnitine uptake. Decreased free carnitine levels discovered by newborn screening has identified many asymptomatic adult women with systemic primary carnitine deficiency (PCD). Here, we presented amplitude integrated electroencephalogram (aEEG) and magnetic resonance imaging (MRI) findings from a neonate with epilepsy whose mother was carnitine deficient. Read More

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https://bmcpediatr.biomedcentral.com/articles/10.1186/s12887
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http://dx.doi.org/10.1186/s12887-019-1452-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6421665PMC
March 2019
5 Reads

LncRNA FTX inhibits hippocampal neuron apoptosis by regulating miR-21-5p/SOX7 axis in a rat model of temporal lobe epilepsy.

Biochem Biophys Res Commun 2019 Apr 11;512(1):79-86. Epub 2019 Mar 11.

Department of Pediatrics, Affiliated Hospital of Yanbian University, Yanji, Jilin, 133000, China. Electronic address:

Emerging evidence has shown that long noncoding RNA (LncRNA) is involved in the development of epileptogenesis. However, the expression profile and the biological function of FTX in epilepsy remains unclear. This study aimed to provide functional evidence and elucidate the molecular mechanisms by which the FTX affects status epilepticus (SE) induced hippocampal apoptosis. Read More

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http://dx.doi.org/10.1016/j.bbrc.2019.03.019DOI Listing

Is it status epilepticus? No, it is infantile tremor syndrome.

Neurol India 2019 Jan-Feb;67(1):332

Department of Pediatrics, Government Medical College and Hospital, Chandigarh, India.

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http://dx.doi.org/10.4103/0028-3886.253615DOI Listing
March 2019
1 Read

End-stage renal disease secondary to anti-glomerular basement membrane disease in a child with common variable immunodeficiency.

Clin Nephrol Case Stud 2019 1;7:1-6. Epub 2019 Feb 1.

Division of Pediatric Nephrology, Department of Pediatrics.

Background: Anti-glomerular basement membrane (GBM) disease is caused by autoantibodies against the α3-chain of type IV collagen in the GBM. Common variable immunodeficiency (CVID) is a primary immunodeficiency manifested by hypogammaglobulinemia, inability to make functional antibody, and recurrent infections. This report extends the phenotype of CVID-associated autoimmune diseases to include anti-GBM disease. Read More

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http://dx.doi.org/10.5414/CNCS109510DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6374989PMC
February 2019
2 Reads

Dravet Syndrome in Lebanon: First Report on Cases with Mutations.

Case Rep Med 2019 21;2019:5270503. Epub 2019 Jan 21.

Institut Jérôme Lejeune, Paris, France.

Dravet syndrome, also known as severe myoclonic epilepsy in infancy, is a rare disease characterized by the appearance of different types of seizures in a healthy baby, triggered by various factors and stressful events. We report 8 Lebanese cases referred for molecular analysis of the gene. Results were positive in 7 cases and revealed de novo variants at the heterozygous state in different exons of the gene for all except one, where the variant was intronic. Read More

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http://dx.doi.org/10.1155/2019/5270503DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6360541PMC
January 2019
1 Read

Acute encephalopathy with brain swelling.

Brain Dev 2019 Feb 22. Epub 2019 Feb 22.

Pediatric Neurology Division, Department of Pediatrics, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Electronic address:

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http://dx.doi.org/10.1016/j.braindev.2019.02.006DOI Listing
February 2019
2 Reads

High-Fidelity Simulation Scenario: Pyridoxine-Dependent Epilepsy and Treatment.

MedEdPORTAL 2018 Sep 21;14:10753. Epub 2018 Sep 21.

Pediatric Emergency Medicine Fellow, Department of Emergency Medicine, State University of New York Upstate Medical University.

Introduction: Treatment of seizures in the neonatal patient is urgent and time sensitive. Effective and timely treatment of this life-threatening condition is vital in preventing mortality and long-term morbidity. This simulation-based curriculum involves the identification and management of a seizure in a 4-day-old neonate with pyridoxine-dependent epilepsy. Read More

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http://dx.doi.org/10.15766/mep_2374-8265.10753DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6342356PMC
September 2018
1 Read

Treatment of Elizabethkingia meningoseptica Neonatal Meningitis with Combination Systemic and Intraventricular Therapy.

Indian J Pediatr 2019 Apr 21;86(4):379-381. Epub 2019 Feb 21.

Department of Pediatrics, Kokilaben Dhirubhai Ambani Hospital and Medical Research Institute, Four Bungalows, Andheri West, Mumbai, 400053, India.

Elizabethkingia meningoseptica, a gram negative bacillus ubiquitous in the hospital environment, is known to infrequently cause serious neonatal infections, particularly meningitis which is associated with high mortality and neuromorbidity in survivors. The authors describe a healthy term newborn with no apparent risk factors who developed Elizabethkingia meningoseptica sepsis and meningitis on day 6 of life. Diagnosis could be established only after a week of the illness by which time the baby developed refractory status epilepticus, ventriculitis and hydrocephalus. Read More

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http://dx.doi.org/10.1007/s12098-019-02875-xDOI Listing
April 2019
1 Read

Acute Encephalopathy With Biphasic Seizures and Late Reduced Diffusion Associated With Adenoviral Pneumonia.

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

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http://dx.doi.org/10.1177/2329048X19826288DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6365989PMC
February 2019
1 Read

Seizure and epilepsy publication in nonneurology journals.

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

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http://dx.doi.org/10.1016/j.yebeh.2019.01.032DOI Listing
February 2019
3 Reads

Functional deficiency in endogenous interleukin-1 receptor antagonist in patients with febrile infection-related epilepsy syndrome.

Ann Neurol 2019 Apr 8;85(4):526-537. Epub 2019 Mar 8.

Department of Neurology, Mayo Clinic, Rochester, MN.

Objective: We recently reported successful treatment of a child with febrile infection-related epilepsy syndrome (FIRES), a subtype of new onset refractory status epilepticus, 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 cerebrospinal fluid (CSF). Read More

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http://dx.doi.org/10.1002/ana.25439DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6450741PMC

Status Epilepticus in the Neonate: Updates in Treatment Strategies.

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

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http://dx.doi.org/10.1007/s11940-019-0546-5DOI Listing
February 2019

Functional brain connectivity in electrical status epilepticus in sleep.

Epileptic Disord 2019 Feb;21(1):55-64

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

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http://dx.doi.org/10.1684/epd.2019.1027DOI Listing
February 2019
3 Reads

Use of Continuous EEG Monitoring in Children Presenting With Encephalopathy Following Convulsive Status Epilepticus.

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

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http://dx.doi.org/10.1097/WNP.0000000000000566DOI Listing
January 2019
7 Reads

Factors affecting epilepsy prognosis in patients with tuberous sclerosis.

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

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http://link.springer.com/10.1007/s00381-019-04066-7
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http://dx.doi.org/10.1007/s00381-019-04066-7DOI Listing
March 2019
11 Reads

PLPHP deficiency: clinical, genetic, biochemical, and mechanistic insights.

Brain 2019 Mar;142(3):542-559

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

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https://academic.oup.com/brain/advance-article/doi/10.1093/b
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http://dx.doi.org/10.1093/brain/awy346DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6391652PMC
March 2019
42 Reads

Dilemmas and Challenges in Treating Seronegative Autoimmune Encephalitis in Indian Children.

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

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http://dx.doi.org/10.4103/ijccm.IJCCM_98_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6311983PMC
December 2018
5 Reads

Sudden unexpected death in GEFS+ families with sodium channel pathogenic variants.

Epilepsy Res 2019 02 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

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http://dx.doi.org/10.1016/j.eplepsyres.2019.01.009DOI Listing
February 2019
5 Reads

The ketogenic diet for super-refractory status epilepticus patients in intensive care units.

Brain Dev 2019 May 9;41(5):420-427. 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

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http://dx.doi.org/10.1016/j.braindev.2018.12.007DOI Listing
May 2019
1 Read
1.542 Impact Factor

The Protective Role of Peroxisome Proliferator-Activated Receptor-Gamma in Seizure and Neuronal Excitotoxicity.

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

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http://dx.doi.org/10.1007/s12035-018-1457-2DOI Listing
January 2019
4 Reads
5.137 Impact Factor

Epilepsy in Children: From Diagnosis to Treatment with Focus on Emergency.

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

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http://www.mdpi.com/2077-0383/8/1/39
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http://dx.doi.org/10.3390/jcm8010039DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6352402PMC
January 2019
15 Reads

Successful use of pure cannabidiol for the treatment of super-refractory status epilepticus.

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

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http://dx.doi.org/10.1016/j.ebcr.2018.07.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6306515PMC
July 2018
2 Reads

Valproic acid and epilepsy: from molecular mechanisms to clinical evidences.

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

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http://dx.doi.org/10.2174/1570159X17666181227165722DOI Listing
December 2018
24 Reads

Identification of a Loss-of-Function Mutation in the Context of Glutaminase Deficiency and Neonatal Epileptic Encephalopathy.

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

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http://archneur.jamanetwork.com/article.aspx?doi=10.1001/jam
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http://dx.doi.org/10.1001/jamaneurol.2018.2941DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439720PMC
December 2018
12 Reads

Sodium valproate suppresses abnormal neurogenesis induced by convulsive status epilepticus.

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

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http://dx.doi.org/10.4103/1673-5374.245475DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6334603PMC
March 2019
2 Reads

Hemiconvulsion-Hemiplegia-Epilepsy in a girl with cobalamin C deficiency.

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

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http://www.john-libbey-eurotext.fr/medline.md?doi=10.1684/ep
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http://dx.doi.org/10.1684/epd.2018.1017DOI Listing
December 2018
8 Reads

KCNQ2 mutation in an infant with encephalopathy of infancy with migrating focal seizures.

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

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http://dx.doi.org/10.1684/epd.2018.1011DOI Listing
December 2018
3 Reads

Nonconvulsive Seizure Detection by Reduced-Lead Electroencephalography in Children with Altered Mental Status in the Emergency Department.

J Pediatr 2019 Apr 7;207:213-219.e3. 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

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http://dx.doi.org/10.1016/j.jpeds.2018.11.019DOI Listing
April 2019
4 Reads

Conventional and quantitative EEG in status epilepticus.

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

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http://dx.doi.org/10.1016/j.seizure.2018.09.011DOI Listing
October 2018
7 Reads

Treatment options in pediatric super-refractory status epilepticus.

Brain Dev 2019 Apr 7;41(4):359-366. 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

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http://dx.doi.org/10.1016/j.braindev.2018.11.011DOI Listing
April 2019
2 Reads

Cannabis-based products for pediatric epilepsy: A systematic review.

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

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http://doi.wiley.com/10.1111/epi.14608
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http://dx.doi.org/10.1111/epi.14608DOI Listing
January 2019
19 Reads

Novel drugs and early polypharmacotherapy in status epilepticus.

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

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http://dx.doi.org/10.1016/j.seizure.2018.08.004DOI Listing
August 2018
3 Reads

Characterisation of neonatal seizures and their treatment using continuous EEG monitoring: a multicentre experience.

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

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http://dx.doi.org/10.1136/archdischild-2018-315624DOI Listing
November 2018
18 Reads

Efficacy, safety, and pharmacokinetics of intravenous midazolam in Japanese children with status epilepticus.

J Neurol Sci 2019 01 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

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https://linkinghub.elsevier.com/retrieve/pii/S0022510X183041
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http://dx.doi.org/10.1016/j.jns.2018.09.035DOI Listing
January 2019
21 Reads

50 Years Ago in The Journal of Pediatrics: The Treatment of Prolonged Seizure Activity with Intravenous Diazepam.

Authors:
Philip Toltzis

J Pediatr 2018 12;203:279

Rainbow Babies and Children's Hospital Cleveland, Ohio.

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http://dx.doi.org/10.1016/j.jpeds.2018.06.017DOI Listing
December 2018

Protocol-Driven Management of Convulsive Status Epilepticus at a Tertiary Children's Hospital: A Quality Improvement Initiative.

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

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http://dx.doi.org/10.1097/PCC.0000000000001816DOI Listing
January 2019
22 Reads

DNM1 Mutation in a child associated with progressive bilateral mesial temporal sclerosis.

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

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http://dx.doi.org/10.1002/ccr3.1793DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6230638PMC
November 2018
1 Read

Immunotherapy for anti-NMDA receptor encephalitis: Experience from a single center in Taiwan.

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

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https://linkinghub.elsevier.com/retrieve/pii/S18759572183035
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http://dx.doi.org/10.1016/j.pedneo.2018.10.006DOI Listing
October 2018
16 Reads

Critical Care in the Pediatric Emergency Department.

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

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https://linkinghub.elsevier.com/retrieve/pii/S00313955183010
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http://dx.doi.org/10.1016/j.pcl.2018.07.004DOI Listing
December 2018
24 Reads

Electroencephalography in Pediatric Epilepsy.

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

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October 2018
4 Reads

Calpain activation and neuronal death during early epileptogenesis.

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

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http://dx.doi.org/10.1016/j.nbd.2018.11.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6381999PMC
April 2019
2 Reads