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J Clin Med 2021 Mar 2;10(5). Epub 2021 Mar 2.

Department of Neurology, University Hospital Frankfurt, Goethe University, 60528 Frankfurt, Germany.

Local anesthetics are commonly administered by nuchal infiltration to provide a temporary interscalene brachial plexus block (ISB) in a surgical setting. Although less commonly reported, local anesthetics can induce central nervous system toxicity. In this case study, we present three patients with acute central nervous system toxicity induced by local anesthetics applied during ISB with emphasis on neurological symptoms, key neuroradiological findings and functional outcome. Read More

Eur J Neurol 2021 Mar 30. Epub 2021 Mar 30.

Neurology Department, Coimbra University and Hospital Centre, Coimbra, Portugal.

Introduction: Creutzfeldt-Jakob disease (CJD) is a rare form of rapidly progressive neurodegenerative disorder. Seizures are uncommon in the early stage of CJD, increasing diagnostic difficulty. We report an autopsy-proven case of CJD presenting initially as an epilepsia partialis continua (EPC), in which the initial workup was all unremarkable. Read More

Brain 2021 Mar 9. Epub 2021 Mar 9.

Department of Psychology, University of California Los Angeles, 3423 Franz Hall, Los Angeles, California 90095, USA.

A common observation in EEG research is that consciousness vanishes with the appearance of delta (1 - 4 Hz) waves, particularly when those waves are high amplitude. High amplitude delta oscillations are very frequently observed in states of diminished consciousness, including slow wave sleep, anaesthesia, generalised epileptic seizures, and disorders of consciousness such as coma and vegetative state. This strong correlation between loss of consciousness and high amplitude delta oscillations is thought to stem from the widespread cortical deactivation that occurs during the "down states" or troughs of these slow oscillations. Read More

Curr Opin Neurol 2021 Apr;34(2):172-181

Department of Clinical Neuroscience, Lausanne University Hospital and University of Lausanne, Lausanne.

Purpose Of Review: Randomized controlled trials investigating the initial pharmacological treatment of status epilepticus have been recently published. Furthermore, status epilepticus arising in comatose survivors after cardiac arrest has received increasing attention in the last years. This review offers an updated assessment of status epilepticus treatment in these different scenarios. Read More

Epileptic Disord 2021 Feb;23(1):191-200

Department of Clinical Neurophysiology, Necker-Enfants Malades Hospital, APHP, Paris, France.

Epileptic myoclonus (EM) is reported in many paediatric epilepsies from neonatal period to adolescence. Myoclonus can be the only seizure type or may occur among others, independently or in combination as a single ictal event. We report two children presenting with absences associated with myoclonus, predominating on one side, in a setting of two different types of absence seizures and two different electro-clinical syndromes. Read More

Epileptic Disord 2021 Feb;23(1):148-152

Kork Epilepsy Center, Kehl-Kork, Germany, Department of Neurology, University Medical Center, Freiburg, Germany.

With an elimination half-life of 105 hours, perampanel (PER) allows a once-daily dosing regimen. In pivotal trials, when PER was tapered, it was therefore usually discontinued abruptly. Thus, in our hospital we have always practiced abrupt cessation. Read More

Brain Dev 2021 Feb 14. Epub 2021 Feb 14.

Center for Integrated Human Brain Science, Brain Research Institute, University of Niigata, Japan. Electronic address:

Background: Acute encephalopathy with acute brain swelling (ABS) is a recently proposed disease of unknown etiology, characterized by rapid progression to whole-brain swelling. To our knowledge, we reported the first case of a patient with acute encephalopathy with ABS wherein brain magnetic resonance imaging (MRI) abnormalities were noted prior to the diffuse brain swelling onset.

Case Presentation: An 11-year-old boy was admitted to our unit owing to prolonged disturbance of consciousness following febrile status epilepticus. Read More

Epilepsy Behav 2021 Mar 4;116:107793. Epub 2021 Feb 4.

Department of Pediatric Neurology, Istanbul Medipol University Faculty of Medicine, Istanbul, Turkey.

Objective: It is argued that early and adequate treatment of electrical status epilepticus in sleep (ESES) is essential to preserve cognitive functions and possibly recovering lost skills. Although antiepileptic drugs (AEDs) are effective in ESES, there is not much experience in the use of sulthiame. In this study, we aimed to examine the efficiency and tolerability of sulthiame in ESES. Read More

J Pediatr 2021 Jan 20. Epub 2021 Jan 20.

Department of Pediatrics & Neurology, University of Colorado, Aurora, CO.

Objectives: To determine how continuous spike and wave during slow wave sleep (CSWS) is currently managed and to compare the effectiveness of current treatment strategies using a database from 11 pediatric epilepsy centers in the US.

Study Design: This retrospective study gathered information on baseline clinical characteristics, CSWS etiology, and treatment(s) in consecutive patients seen between 2014 and 2016 at 11 epilepsy referral centers. Treatments were categorized as benzodiazepines, steroids, other antiseizure medications (ASMs), or other therapies. Read More

Epilepsy Res 2021 Feb 5;170:106549. Epub 2021 Jan 5.

University of Health Sciences, Dr. Sami Ulus Training and Research Hospital, Department of Pediatric Neurology, Ankara, Turkey. Electronic address:

Purpose: This study aimed to describe the electroclinical spectrum and neurocognitive outcome in children with epileptic encephalopathy with status epilepticus during sleep (ESES) according to the EEG patterns.

Methods: Records of 48 (19 males, 29 females) patients with ESES/CSWS syndrome were retrospectively evaluated for data on sleep and awake EEGs, psychometric tests, and brain MRI. Patients with a spike-wave index (SWI) of at least 50 % in the NREM sleep EEG were included in the study. Read More

World J Clin Cases 2020 Dec;8(24):6480-6486

Liver Transplantation Center, National Clinical Research Center for Digestive Diseases, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.

Background: Status epilepticus in patients with hepatic encephalopathy (HE) is a rare but serious condition that is refractory to antiepileptic drugs, and current treatment plans are vague. Diagnosis may be difficult without a clear history of cirrhosis. Liver transplantation (LT) is effective to alleviate symptoms, however, there are few reports about LT in the treatment of status epilepticus with HE. Read More

J Neurol Sci 2021 01 26;420:117279. Epub 2020 Dec 26.

Department of Neurology, Louisiana State University School of Medicine, New Orleans, USA.

Background: The Chikungunya Virus (CHIKV) was introduced into Honduras in 2015. Since then the WHO has reported more than 14,000 suspected cases in the country.

Objective: To describe the clinical, laboratory, neuroimaging, and pathological features of CHIKV encephalitis. Read More

Int J Neural Syst 2021 Apr 23;31(4):2150002. Epub 2020 Dec 23.

The Clinical Hospital of Chengdu Brain Science Institute, MOE Key Lab for NeuroInformation, Center for Information in Medicine, School of Life Science and Technology, University of Electronic, Science and Technology of China, Chengdu Sichuan 611731, P. R. China.

Very fast ripples (VFRs, 500-1000[Formula: see text]Hz) are considered more specific than high-frequency oscillations (80-500[Formula: see text]Hz) as biomarkers of epileptogenic zones. Although VFRs are frequent abnormal phenomena in epileptic seizures, their functional roles remain unclear. Here, we detected the VFRs in the hippocampal network and tracked their roles during status epilepticus (SE) in rats with pilocarpine-induced temporal lobe epilepsy (TLE). Read More

Clin Neurophysiol 2021 Jan 13;132(1):146-153. Epub 2020 Nov 13.

Department of Clinical Neurosciences, Cumming School of Medicine, University of Calgary, Calgary AB T2N 1N4, Canada; Department of Pediatrics, Section of Neurology, Cumming School of Medicine, University of Calgary, Calgary AB T2N 1N4, Canada; Alberta Children's Hospital, Calgary AB T3B 6A8, Canada. Electronic address:

Objective: Continuous spike and wave in slow-wave sleep (CSWS), an epileptic encephalopathy, occurs after perinatal stroke where it is associated with cognitive decline. CSWS features a distinct EEG pattern, electrical status epilepticus in sleep (ESES). Biomarkers for the prediction of ESES have not been identified but will facilitate earlier diagnosis and treatment. Read More

Clin Neurophysiol 2021 Jan 17;132(1):114-125. Epub 2020 Nov 17.

Clinical Neurophysiology Lab, Centro Hospitalar Psiquiátrico de Lisboa, Lisbon, Portugal; EEG Lab - Pediatric Neurology Department, Hospital Dona Estefânia, Lisbon, Portugal. Electronic address:

Objective: Encephalopathy with Status Epilepticus during slow Sleep (ESES) is a syndrome where neurocognitive impairment correlates with multifocal Electroencephalography (EEG) spikes increasing abruptly at sleep onset. Demonstration of a focal onset could provide important clues to unravel the mechanisms underlying the condition, but until know it has not been established.

Methods: We studied epileptic dynamics at sleep onset to assess its focal or diffuse features in five patients with perinatal thalamic hemorrhages lateralized to one hemisphere, using high resolution EEG. Read More

Seizure 2021 Jan 17;84:34-39. Epub 2020 Nov 17.

Unité de Neurologie et de Neurochirurgie de l'enfant, service de pédiatrie, CHU Angers, France; Laboratoire Angevin de Recherche en Ingénierie des Systèmes (LARIS), University of Angers, France.

Purpose: To determine whether awake EEG criteria can differentiate epileptic encephalopathy with continuous spike and waves during sleep (EE-CSWS) at the time of cognitive regression from typical, self-limited focal epilepsy (SFE).

Methods: This retrospective case-control study was based on the analysis of awake EEGs and included 15 patients with EE-CSWS and 15 age-matched and sex-matched patients with typical SFE. The EEGs were anonymised and scored by four independent readers. Read More

Int J Neurosci 2020 Dec 9:1-3. Epub 2020 Dec 9.

Dietician, University of Health Sciences, Dr. Behcet Uz Children's Training and Research Hospital, Izmir, Turkey.

Purpose/aim: Ehlers-Danlos syndrome (EDS) is a hereditary connective tissue disease. Epilepsy is not a common neurological finding in EDS. Here we report a pediatric patient with EDS comorbid with related epileptic encephalopathy as 'electrical status epilepticus during slow-wave sleep (ESES)' and whose refractory epileptic seizures were controlled with ketogenic diet. Read More

Front Pediatr 2020 6;8:574803. Epub 2020 Nov 6.

Department of Neurology, Children's Hospital of Chongqing Medical University, Chongqing, China.

This study aims to analyze the electroclinical characteristics and gene test results of children on the severe end of the epilepsy aphasia spectrum (EAS) and also the correlation of EAS-related GRIN2A genes to explore the genotype-phenotype relationships, as well as potential pathogenic mechanism of EAS. A retrospective study was conducted on the participants diagnosed with Landau-Kleffner syndrome (LKS), epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS), and atypical benign partial epilepsy (ABPE) at the Children's Hospital of Chongqing Medical University from January 2013 to June 2019. Whole-exome sequencing was performed in six patients, and epileptic panel was carried out in two. Read More

Eur J Paediatr Neurol 2021 Jan 24;30:121-127. Epub 2020 Oct 24.

Division of Paediatric Epileptology, Centre for Paediatrics and Adolescent Medicine, University Hospital Heidelberg, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany. Electronic address:

Objective: Continuous spikes and waves during sleep (CSWS) is an epileptic encephalopathy characterized by generalised epileptiform activity and neurocognitive dysfunction. Causes and outcome are diverse and treatment is mainly empirical.

Methods: Retrospective descriptive analysis of clinical and EEG data of children with CSWS diagnosed between 1998 and 2018 at the University Hospital Heidelberg. Read More

Brain Sci 2020 Oct 23;10(11). Epub 2020 Oct 23.

Department of Neuroscience, Bambino Gesù Children's Hospital, IRCCS, Full Member of European Reference Network EpiCARE, 00165 Rome, Italy.

Background: The clinical spectrum associated with gene mutations ranges from non-syndromic epilepsy or mild isolated neurological signs to neurodegenerative disorders. Our aim was to review diagnostic findings, therapeutic approaches and outcomes of reported cases of epilepsy related to mutation.

Methods: The articles for review were identified through a systematic research on PubMed and EMBASE databases from January 2003 to April 2020, searching for the terms "Epilepsy AND OR polymerase gamma," OR "". Read More

Seizure 2020 Dec 13;83:1-4. Epub 2020 Oct 13.

Neurology Department, Pontificia Universidad Católica de Chile, Santiago, Chile; Neurology Service, Hospital Dr. Sótero del Río, Santiago, Chile. Electronic address:

Purpose: Our objective is to describe the most prevalent electroencephalographic findings in COVID-19 hospitalized patients, and to determine possible predictors of mortality including EEG and clinical variables.

Methods: A multicentric prospective observational study in patients with COVID-19 requiring EEG during hospitalization.

Results: We found 94 EEG from 62 patients (55 % men, mean age 59. Read More

Neurocrit Care 2020 Oct 6. Epub 2020 Oct 6.

Department of Neurology and Neurological Sciences, Stanford University Medical Center, 300 Pasteur Drive, Stanford, CA, 94305, USA.

Introduction: Current electroencephalography (EEG) practice relies on interpretation by expert neurologists, which introduces diagnostic and therapeutic delays that can impact patients' clinical outcomes. As EEG practice expands, these experts are becoming increasingly limited resources. A highly sensitive and specific automated seizure detection system would streamline practice and expedite appropriate management for patients with possible nonconvulsive seizures. Read More

Front Neurol 2020 28;11:911. Epub 2020 Aug 28.

Institute of Behavioral Sciences, Semmelweis University, Budapest, Hungary.

We aimed to explore the link between NREM sleep and epilepsy. Based on human and experimental data we propose that a sleep-related epileptic transformation of normal neurological networks underlies epileptogenesis. Major childhood epilepsies as medial temporal lobe epilepsy (MTLE), absence epilepsy (AE) and human perisylvian network (PN) epilepsies - made us good models to study. Read More

Ann N Y Acad Sci 2020 11 11;1480(1):183-206. Epub 2020 Sep 11.

Department of Neurology, School of Medicine, University of California, Davis, Sacramento, California.

Combinations of midazolam, allopregnanolone, and perampanel were assessed for antiseizure activity in a rat diisopropylfluorophosphate (DFP) status epilepticus model. Animals receiving DFP followed by atropine and pralidoxime exhibited continuous high-amplitude rhythmical electroencephalography (EEG) spike activity and behavioral seizures for more than 5 hours. Treatments were administered intramuscularly 40 min after DFP. Read More

Brain Dev 2021 Feb 8;43(2):268-279. Epub 2020 Sep 8.

Sackler Faculty of Medicine, Tel-Aviv University, Israel; Pediatric Neurology Institute, Dana-Dwek Children's Hospital, Tel Aviv Sourasky Medical Center, Israel.

Background: Most children with Benign epilepsy with centro-temporal spikes (BECTS) undergo remission during late adolescence and do not require treatment. In a small group of patients, the condition may evolve to encephalopathic syndromes including epileptic encephalopathy with continuous spike-and-wave during sleep (ECSWS), or Landau-Kleffner Syndrome (LKS). Development of prediction models for early identification of at-risk children is of utmost importance. Read More

Seizure 2020 Oct 21;81:250-253. Epub 2020 Aug 21.

Universidade Federal do Ceará, Brazil.

Background: Brazil experienced a disproportionately higher rate of microcephaly cases in November 2015 with evidence of a causal link with Zika virus (ZIKV) infections during pregnancy. Epilepsy is a major neurological feature seen as part of congenital Zika virus syndrome (CZVS). Different seizure types and electroencephalographic (EEG) abnormalities have been described in association with this syndrome. Read More

Int J Mol Sci 2020 Aug 20;21(17). Epub 2020 Aug 20.

Rudolf Boehm Institute for Pharmacology and Toxicology, University of Leipzig, 04107 Leipzig, Germany.

ATP is a (co)transmitter and signaling molecule in the CNS. It acts at a multitude of ligand-gated cationic channels termed P2X to induce rapid depolarization of the cell membrane. Within this receptor-channel family, the P2X7 receptor (R) allows the transmembrane fluxes of Na, Ca, and K, but also allows the slow permeation of larger organic molecules. Read More

Seizure 2020 08 3;80:257-258. Epub 2020 Jul 3.

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

Rev Neurosci 2020 10;31(7):793-802

Department of Pediatrics, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, 430030, China.

Encephalopathy related to Status Epilepticus during slow Sleep (ESES) is an age-dependent syndrome characterized by the appearance of neuropsychological and behavioral disorders associated with extreme activation of epileptic activity during sleep. The major goal of therapy in ESES is to prevent neuropsychological deficits. Effective therapy to reduce seizures and resolve the EEG pattern of status epilepticus during sleep (SES) may be crucial to improve long-term prognosis. Read More

J Neural Eng 2020 08 11;17(4):046032. Epub 2020 Aug 11.

Center for Intelligent Medical Electronics (CIME), School of Information Science and Engineering, Fudan University, Shanghai, People's Republic of China. Xian Zhao and Xinhua Wang contributed equally to this work.

Objective: Electrical status epilepticus during sleep (ESES), as electroencephalographic disturbances, is characterized by strong activation of epileptiform activity in the electroencephalogram during sleep. Quantitative descriptors of such epileptiform activity can support the diagnose and the prognosis of children with ESES. To quantify the epileptiform activity of ESES, a knowledge-based approach to mimic the clinical decision-making process is proposed. Read More