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Epilepsia 2022 May 3. Epub 2022 May 3.

Robert Debré Hospital, Public Hospital Network of Paris, NeuroDiderot, National Institute of Health and Medical Research, Department Medico-Universitaire Innovation Robert-Debré, Pediatric Neurology, University of Paris, Paris, France.

The 2017 International League Against Epilepsy classification has defined a three-tier system with epilepsy syndrome identification at the third level. Although a syndrome cannot be determined in all children with epilepsy, identification of a specific syndrome provides guidance on management and prognosis. In this paper, we describe the childhood onset epilepsy syndromes, most of which have both mandatory seizure type(s) and interictal electroencephalographic (EEG) features. Read More

World Neurosurg 2022 Apr 30;164:69. Epub 2022 Apr 30.

Neurodynamics Laboratory and Harvard Medical School, Boston, Massachusetts, USA; Division of Epilepsy Surgery, Department of Neurosurgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA. Electronic address:

Corpus callosotomy is an interhemispheric disconnection by callosal commissural fiber ablation. Its rationale is the disruption of ictal spread to prevent seizure generalization. The objective pursued is alleviation of intractable, debilitating, and injurious manifestations of generalized epilepsy. Read More

JAMA Neurol 2022 May 2. Epub 2022 May 2.

Department of Neurology, Epilepsy Program, Hospital Ruber Internacional, Madrid, Spain.

Importance: New treatment options are needed for patients with Lennox-Gastaut syndrome (LGS), a profoundly impairing, treatment-resistant, developmental and epileptic encephalopathy.

Objective: To evaluate the efficacy and safety of fenfluramine in patients with LGS.

Design, Setting, And Participants: This multicenter, double-blind, placebo-controlled, parallel-group randomized clinical trial was conducted from November 27, 2017, to October 25, 2019, and had a 20-week trial duration. Read More

Rev Neurol (Paris) 2022 Apr 27. Epub 2022 Apr 27.

Department of Pediatric Neurology, CHU d'Angers, and Laboratoire Angevin de Recherche en Ingénierie des Systèmes (LARIS), Université d'Angers, 4, rue Larrey, 49000 Angers, France. Electronic address:

Developmental and epileptic encephalopathies are conditions where there is developmental impairment related to both the underlying etiology independent of epileptiform activity and the epileptic encephalopathy. Usually they have multiple etiologies. Therefore, long-term outcome is related to both etiology-related factors and epilepsy-related factors-age at onset of epilepsy, type(s) of seizure(s), type of electroencephalographic abnormalities, duration of the epileptic disorder. Read More

Seizure 2022 Apr 9. Epub 2022 Apr 9.

Department of Pharmacy Practice, National Institute of Pharmaceutical Education and Research, SAS Nagar, Punjab 160062, India. Electronic address:

Purpose: To assess the short-term and long-term comparative efficacy and safety of ASMs for Lennox-Gastaut syndrome (LGS).

Methods: Following a systematic literature search, randomized controlled trial (RCT) and open-label extension (OLE) studies on LGS comparing ASMs with placebo or other ASMs were included. ≥50% reduction in drop seizure frequency from baseline and occurrence of treatment-emergent adverse events (TEAEs) were the primary efficacy and safety outcomes. Read More

Neurosciences (Riyadh) 2022 Apr;27(2):59-64

From the Department of Adult Neurology (Al-Attas), Pediatric Neurology Department (Alanazi, Al Osaimi, Alwadei, Al-Otaibi, Jad), National Neuroscience Institute, King Fahad Medical City, Riyadh, Kingdom of Saudi Arabia.

Objectives: To compare the efficacy and safety of corpus callosotomy versus vagus nerve stimulation (VNS) as long-term adjunctive therapies in children with Lennox-Gastaut syndrome.

Methods: This retrospective study was conducted in King Fahad Medical City between 2010 and 2019. The authors identified and followed 9 patients with Lennox-Gastaut syndrome (LGS) who underwent corpus callosotomy or VNS implantation for at least 12 months; seizure frequency and major complications were monitored. Read More

J Clin Neurosci 2022 Jun 22;100:138-142. Epub 2022 Apr 22.

Epilepsy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

The aim of the current study was to investigate the age at onset in patients with Lennox-Gastaut Syndrome (LGS). We also investigated the clinical and EEG characteristics of these patients in different age groups. This was a retrospective study. Read More

Ann Neurol 2022 Apr 15. Epub 2022 Apr 15.

Department of Medicine (Austin Health), University of Melbourne, Heidelberg, Vic, Australia.

Objective: Deep brain stimulation (DBS) can reduce seizures in Lennox-Gastaut syndrome (LGS). However, little is known about the optimal target and whether efficacy depends on connectivity of the stimulation site. Using outcome data from the ESTEL trial, we aimed to determine the optimal target and connectivity for DBS in LGS. Read More

Ann Neurol 2022 Apr 12. Epub 2022 Apr 12.

J Neurosurg 2022 Apr 8:1-10. Epub 2022 Apr 8.

Departments of1Neurosurgery and.

Objective: Neuromodulation of the centromedian nucleus of the thalamus (CM) has unclear effectiveness in the treatment of drug-resistant epilepsy. Prior reports suggest that it may be more effective in the generalized epilepsies such as Lennox-Gastaut syndrome (LGS). The objective of this study was to determine the outcome of CM deep brain stimulation (DBS) at the authors' institution. Read More

Expert Opin Emerg Drugs 2022 Mar 1;27(1):75-90. Epub 2022 Apr 1.

Mid-Atlantic Epilepsy and Sleep Center, Comprehensive Neurology Clinics of Bethesda, Bethesda, MD, USA.

Introduction: Despite the existence of over 30 anti-seizure medications (ASM), including 20 over the last 30 years, a third of patients with epilepsy remain refractory to treatment, with no disease-modifying or preventive therapies until very recently. The development of new ASMs with new mechanisms of action is therefore critical. Recent clinical trials of new treatments have shifted focus from traditional common epilepsies to rare, genetic epilepsies with known mechanistic targets for treatment and disease-specific animal models. Read More

Dev Med Child Neurol 2022 Jun 23;64(6):678-680. Epub 2022 Mar 23.

Wake Forest School of Medicine, Winston-Salem, NC, USA.

Epilepsy Res 2022 May 16;182:106911. Epub 2022 Mar 16.

Department of Neuroscience, Aziz Sancar Institute of Experimental Medicine, Istanbul University, Istanbul, Turkey. Electronic address:

Background: Neuronal autoantibodies and favorable response to immunosuppressive treatment have been described in patients with chronic epilepsy of unknown cause, suggesting autoimmune etiology. Our aim was to identify novel epilepsy-specific autoantibodies reactive with neuronal surface antigens.

Methods: Sera of 172 epilepsy patients with unknown cause and 30 healthy controls were screened with indirect immunofluorescence to identify IgG reacting with primary rat neuronal cultures. Read More

J Korean Med Sci 2022 Mar 14;37(10):e82. Epub 2022 Mar 14.

Department of Pediatrics, Jeonbuk National University Children's Hospital, Jeonju, Korea.

The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) resulted in the coronavirus disease 2019 (COVID-19) pandemic. COVID-19 can result in fatal comorbidities, including acute respiratory distress syndrome (ARDS). Several reports suggest that children have milder illness, though severe cases have still been reported. Read More

Ann Clin Transl Neurol 2022 Apr 10;9(4):497-505. Epub 2022 Mar 10.

The Center for Discovery, Harris, New York, USA.

Objectives: Medical cannabis formulations with cannabidiol (CBD) and delta-9-tetrahydrocannabinol (THC) are widely used to treat epilepsy. We studied the safety and efficacy of two formulations.

Methods: We prospectively observed 29 subjects (12 to 46 years old) with treatment-resistant epilepsies (11 Lennox-Gastaut syndrome; 15 with focal or multifocal epilepsy; three generalized epilepsy) were treated with medical cannabis (1THC:20CBD and/or 1THC:50CBD; maximum of 6 mg THC/day) for ≥24 weeks. Read More

Neurol India 2022 Jan-Feb;70(1):63-67

Department of Neurology, All India Institute of Medical Sciences (AIIMS), New Delhi, India.

Background: Corpus callosotomy (CC) is a major disconnection procedure that functionally isolates the cerebral hemispheres, thereby interrupting the spread of epileptic activity from one hemisphere to the other. It is extremely useful in children suffering from non-localized drug refractory epilepsy, especially drop attacks. The technique has evolved from microscopic to minimally invasive endoscopic surgery. Read More

Seizure 2022 Mar 6;96:137-141. Epub 2022 Feb 6.

Department of Neurosurgery, Medical College of Wisconsin, Milwaukee, WI, USA.

Purpose: The Laser interstitial thermal therapy (LITT) technique has been used recently for corpus callosotomy in patients with epilepsy, especially atonic seizures (drop attacks) and Lennox-Gastaut Syndrome (LGS). However, there is little data on safety and outcomes. Therefore, the authors systematically studied and conducted a pooled analysis with special focus on feasibility, outcomes and complications. Read More

Front Genet 2022 11;13:761178. Epub 2022 Feb 11.

Faculty of Pediatrics, Chinese PLA General Hospital, BaYi Children's Hospital, The Seventh Medical Center of PLA General Hospital, Beijing, China.

Pathogenic variants in have been reported to have a wide range of phenotypic variability in neurodevelopmental disorders, such as early-onset epileptic encephalopathy, developmental delay, and behavior problems. So far, there is no clear correlation between genotypes and phenotypes. This study reports a Chinese patient with a novel heterozygous mutation (c. Read More

Neurol Neurochir Pol 2022 25;56(1):14-20. Epub 2022 Feb 25.

Department of Developmental Neurology, Medical University of Gdansk, Poland.

Introduction: Cannabis sativa has been cultivated for human use for about 5,000 years, and has likewise been used in the treatment of epilepsy for thousands of years.

State Of The Art: Cannabidiol (CBD), which was isolated from cannabis sativa in 1940, has an anti-seizure effect and no psychoactive activity. Its effectiveness in reducing various types of seizures has been proven in animal seizure and epilepsy models. Read More

Epilepsy Behav 2022 03 11;128:108586. Epub 2022 Feb 11.

Department of Neurology, Children's Hospital of Colorado, Aurora, CO, United States.

Objective: Developmental and epileptic encephalopathies (DEE) entail moderate to profound communication and other impairments that are poorly measured by typical clinical outcomes assessments (COA). We examined the potential of alternative approaches, specifically, the use of raw scores and COAs outside of their intended age ranges.

Methods: In a cross-sectional survey, 120 parents of children with Dravet Syndrome, Lennox-Gastaut syndrome, KCNQ2-DEE, KCNB1-DEE, and SCN2A-DEE (ages 1-35 years) completed the Adaptive Behavior Assessment System-3 for ages 0-5 years, modified checklist for autism (mCHAT), communication and social behavior scales (CSBS), communication matrix (CM), and several parent-reported classifiers of communication. Read More

J Neurosci Rural Pract 2022 Jan 7;13(1):32-42. Epub 2022 Jan 7.

Department of Pediatrics, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.

 Lennox-Gastaut syndrome (LGS) is one of the most difficult to treat childhood-onset epileptic encephalopathies. There is growing evidence that lacosamide is safe and efficacious in patients and adults with refractory epilepsy. However, the evidence regarding the efficacy of lacosamide in LGS is controversial so far. Read More

Curr Opin Neurol 2022 04;35(2):145-154

Division of Child and Adolescent Neurology, Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.

Purpose Of Review: Summarize evidence on Developmental and Epileptic Encephalopathies (DEEs) treatments focusing on new and emerging pharmacologic therapies (see Video, http://links.lww.com/CONR/A61, Supplementary Digital Content 1, which provides an overview of the review). Read More

Pediatr Int 2022 Jan;64(1):e14871

Department of Pediatrics, Toho University, Sakura, Chiba, Japan.

Stem Cell Res 2022 04 15;60:102667. Epub 2022 Jan 15.

Department of Neurology, Guangzhou Women and Children's Medical Center, Guangzhou Medical University, 9# Jin Sui Road, Guangzhou 510623, China. Electronic address:

Lennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy, which may arise from genetic etiologies. TANC2 mutation may cause the neurodevelopmental disorders and epilepsy such as LGS. By reprogramming with SOX2, KLF4, c-MYC and OCT3/4, we generated an induced pluripotent stem cell (iPSC) line from peripheral blood mononuclear cells (PBMCs) collected from a two-year-old Chinese boy individual to be used as a disease model with TANC2 mutation. Read More

Biochem Pharmacol 2022 03 19;197:114928. Epub 2022 Jan 19.

Department of Physiology, National Taiwan University College of Medicine, Taipei, Taiwan; Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan. Electronic address:

Na channels undergo multiple inactivated states with different kinetics, which set the refractory period of neuronal discharges, but isolating the intermediate inactivated state has been challenging. Most classical Nachannel-inhibiting anticonvulsants bind to the fast inactivated state to reduce Nacurrents and cellular excitability. These anticonvulsants have the slow binding kinetics and thus necessitate long depolarization for drug action, a "use-dependent" effect sparing most normal activities. Read More

Front Neural Circuits 2021 3;15:781113. Epub 2022 Jan 3.

Department of Neurophysiology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.

Research on endocannabinoid signaling has greatly advanced our understanding of how the excitability of neural circuits is controlled in health and disease. In general, endocannabinoid signaling at excitatory synapses suppresses excitability by inhibiting glutamate release, while that at inhibitory synapses promotes excitability by inhibiting GABA release, although there are some exceptions in genetically epileptic animal models. In the epileptic brain, the physiological distributions of endocannabinoid signaling molecules are disrupted during epileptogenesis, contributing to the occurrence of spontaneous seizures. Read More

Epilepsia 2022 03 15;63(3):686-696. Epub 2022 Jan 15.

Department of Pediatrics, Antwerp University Hospital, Edegem, Belgium.

Objective: This study was undertaken to determine the plasma concentration and pharmacokinetic variability of fenfluramine (FFA) and its main active metabolite norfenfluramine (norFFA) in relation to the prevalence of adverse effects in patients with refractory epilepsy treated with FFA. In addition, the interaction with concomitant antiseizure medications including stiripentol (STP) is studied.

Methods: Patients were recruited at our center from two open-label sources, an investigator-initiated observational study and an international multicenter extension study. Read More

Front Neurol 2021 24;12:743726. Epub 2021 Dec 24.

Department of Neurosurgery, General Hospital of Ningxia Medical University, Yinchuan, China.

is one of the most common epilepsy genes. About 80% of gene mutations cause Dravet syndrome (DS), which is a severe and catastrophic epileptic encephalopathy. More than 1,800 mutations have been identified in . Read More

Zhonghua Er Ke Za Zhi 2022 Jan;60(1):51-55

Department of Pediatrics, the Third Affiliated Hospital of Zhengzhou University, Zhengzhou 450002, China.

To explore the clinical manifestations and genetic characteristics of patients with epilepsy and episodic ataxia caused by SCN2A gene variation. The clinical data of seizure manifestation, imaging examination and genetic results of 5 patients with epilepsy and (or) episodic ataxia because of SCN2A gene variation admitted to the Department of Pediatrics, the Third Affiliated Hospital of Zhengzhou University from July 2017 to January 2021 were analyzed retrospectively. Among 5 patients, 4 were female and 1 was male. Read More