1,297 results match your criteria Lennox-Gastaut Syndrome


Lennox-Gastaut Syndrome in Mitochondrial Disease.

Yonsei Med J 2019 Jan;60(1):106-114

Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.

Purpose: Previous studies have shown that neurologic symptoms are dominant in patients with mitochondrial diseases, and most of these patients have seizure-related disorders. The epileptic classification of these patients as Lennox-Gastaut syndrome (LGS) is as high as 25%. This study aimed to investigate the clinical manifestations, diagnoses, treatments, and epilepsy in LGS, which is associated with mitochondrial disease. Read More

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https://synapse.koreamed.org/DOIx.php?id=10.3349/ymj.2019.60
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http://dx.doi.org/10.3349/ymj.2019.60.1.106DOI Listing
January 2019
2 Reads

Spectrum and time course of epilepsy and the associated cognitive decline in duplication syndrome.

Neurology 2018 Dec 14. Epub 2018 Dec 14.

From the Departments of Neurology (D.M., B.S., R.S., D.G., V.N.P., A.M.G.) and Pediatrics (R.S., D.G.), Baylor College of Medicine, Houston, TX.

Objective: We characterized the epilepsy features and contribution to cognitive regression in 47 patients with duplication syndrome (MDS) and reviewed these characteristics in over 280 MDS published cases.

Methods: The institutional review board approved this retrospective review of medical records and case histories of patients with MDS.

Results: The average age at enrollment was 10 ± 7 years. Read More

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http://dx.doi.org/10.1212/WNL.0000000000006742DOI Listing
December 2018

The refractory epilepsy screening tool for Lennox-Gastaut syndrome (REST-LGS).

Epilepsy Behav 2018 Dec 8;90:148-153. Epub 2018 Dec 8.

Icahn School of Medicine at Mount Sinai Health Systems, 10 Union Square East, Suite 5G, New York, NY 10003, USA. Electronic address:

Background: The complex clinical presentation and progression of Lennox-Gastaut syndrome (LGS) can complicate the accurate diagnosis of this severe, lifelong, childhood-onset epilepsy, often resulting in suboptimal treatment. The Refractory Epilepsy Screening Tool for LGS (REST-LGS) was developed to improve the identification of patients with LGS.

Methods: Using the Modified Delphi Consensus, a group of experts developed and tested the REST-LGS Case Report Form (CRF) comprising 8 criteria (4 major, 4 minor) considered potentially indicative of LGS. Read More

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http://dx.doi.org/10.1016/j.yebeh.2018.11.016DOI Listing
December 2018
1 Read

Evaluation of WO2014121383 A1: a process for preparation of rufinamide and intermediates.

Expert Opin Ther Pat 2018 Nov 15:1-4. Epub 2018 Nov 15.

a Department of Chemistry , School of Advanced Sciences, Vellore Institute of Technology , Vellore , India.

Introduction: There is great potential in the synthetic development of rufinamide to treat childhood-onset epilepsy known as Lennox-Gastaut syndrome (LGS). Areas covered: 1,4-disubstituted triazole ring formed by 1,3-dipolar cycloaddition reaction is an important structural motif widely used to construct diverse chemotypes in chemical, biological, and material fields. 1,2,3-triazole ring containing rufinamide, an antiepileptic drug developed by Novartis, is useful in combination with other antiepileptic medicaments for the treatment of childhood-onset epilepsy known as LGS. Read More

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https://www.tandfonline.com/doi/full/10.1080/13543776.2019.1
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http://dx.doi.org/10.1080/13543776.2019.1549230DOI Listing
November 2018
1 Read

What is the interest of the electroencephalogram in the syndromic diagnosis?

Tunis Med 2018 Aug - Sep;96(8-09):528-531

Introduction: The identification of the epileptic syndrome is a challenge particularly in childhood epilepsies. In fact, the diagnosis may need several years to be fulfilled.

Observation: Our patient presented at the age of 3 years 6 months atypical absence. Read More

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November 2018
13 Reads

Lennox-Gastaut Syndrome: In a Nutshell.

Cureus 2018 Aug 13;10(8):e3134. Epub 2018 Aug 13.

Internal Medicine, Punjab Medical College Allied Hospital Faisalabad, Faisalabad, PAK.

Lennox-Gastaut syndrome is one of the rare childhood-onset epileptic encephalopathies, characterized by multiple type seizure disorder, the typical pattern on electroencephalogram and intellectual disability. Tonic-type seizures are most commonly seen in these patients. Behavioral disturbances and cognitive decline are gradual-onset and last long after the first episode of epileptiform activity. Read More

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https://www.cureus.com/articles/14127-lennox-gastaut-syndrom
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http://dx.doi.org/10.7759/cureus.3134DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6207167PMC
August 2018
4 Reads

[Ten years' experience with vagus nerve stimulation in a paediatric population].

Rev Neurol 2018 Nov;67(10):382-386

Hospital Infantil Universitario Nino Jesus, 28009 Madrid, Espana.

Introduction: Fifty million people are affected by epilepsy. Up to 30% are not controlled with the aid of antiepileptic drugs. The vagus nerve stimulator (VNS) is a therapeutic alternative that must be taken into account. Read More

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November 2018
4 Reads

Efficacy of cannabinoids in paediatric epilepsy.

Dev Med Child Neurol 2019 Jan 6;61(1):13-18. Epub 2018 Nov 6.

Department of Paediatrics and Child Health, University of Otago, Wellington, New Zealand.

There are hundreds of compounds found in the marijuana plant, each contributing differently to the antiepileptic and psychiatric effects. Cannabidiol (CBD) has the most evidence of antiepileptic efficacy and does not have the psychoactive effects of ∆ -tetrahydrocannabinol. CBD does not act via cannabinoid receptors and its antiepileptic mechanism of action is unknown. Read More

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http://doi.wiley.com/10.1111/dmcn.14087
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http://dx.doi.org/10.1111/dmcn.14087DOI Listing
January 2019
6 Reads

The pharmacological management of Lennox-Gastaut syndrome and critical literature review.

Seizure 2018 Dec 26;63:17-25. Epub 2018 Oct 26.

Department of child Neuropsychiatry, Tor Vergata University of Rome, Rome, Italy. Electronic address:

Lennox-Gastaut syndrome (LGS) is a severe epileptic encephalopathy with a prevalence of 1-2% of all patients with epilepsy. It is characterized by multiple pharmaco-resistant seizure types, including tonic, atypical absences and tonic or atonic drop attacks, and the presence of electroencephalographic abnormalities, such as slow-spike waves and paroxysmal fast rhythms. Intellectual disability, behavioural and psychiatric disorders are common comorbidities; these disturbances have a multi-factorial pathogenesis. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10591311183055
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http://dx.doi.org/10.1016/j.seizure.2018.10.016DOI Listing
December 2018
8 Reads

Efficacy and Safety of Cannabidiol in Epilepsy: A Systematic Review and Meta-Analysis.

Drugs 2018 Nov;78(17):1791-1804

Department of Experimental and Clinical Medicine, Neurological Clinic, Marche Polytechnic University, Via Conca 71, 60020, Ancona, Italy.

Background: Approximately one-third of patients with epilepsy presents seizures despite adequate treatment. Hence, there is the need to search for new therapeutic options. Cannabidiol (CBD) is a major chemical component of the resin of Cannabis sativa plant, most commonly known as marijuana. Read More

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http://dx.doi.org/10.1007/s40265-018-0992-5DOI Listing
November 2018
5 Reads

Medical Use of Cannabinoids.

Drugs 2018 Nov;78(16):1665-1703

Department of Pharmaceutics and Food Technology, Faculty of Pharmacy, Plaza Ramón y Cajal s/n, 28040 , Madrid, Spain.

Cannabinoid receptors, endocannabinoids and the enzymes responsible for their biosynthesis and degradation constitute the endocannabinoid system. In recent decades, the endocannabinoid system has attracted considerable interest as a potential therapeutic target in numerous pathological conditions. Its involvement in several physiological processes is well known, such as in energy balance, appetite stimulation, blood pressure, pain modulation, embryogenesis, nausea and vomiting control, memory, learning and immune response, among others, as well as in pathological conditions where it exerts a protective role in the development of certain disorders. Read More

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http://link.springer.com/10.1007/s40265-018-0996-1
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http://dx.doi.org/10.1007/s40265-018-0996-1DOI Listing
November 2018
10 Reads

Progress report on new antiepileptic drugs: A summary of the Fourteenth Eilat Conference on New Antiepileptic Drugs and Devices (EILAT XIV). II. Drugs in more advanced clinical development.

Epilepsia 2018 Oct;59(10):1842-1866

Department of Pharmacy, School of Pharmacy, University of Washington, Seattle, Washington.

The Fourteenth Eilat Conference on New Antiepileptic Drugs and Devices (EILAT XIV) took place in Madrid, Spain, on May 13-16, 2018 and was attended by 168 delegates from 28 countries. The conference provided a forum for professionals involved in basic science, clinical research, regulatory affairs, and clinical care to meet and discuss the latest advances related to discovery and development of drugs and devices aimed at improving the management of people with epilepsy. This progress report provides a summary of findings on investigational compounds for which data from both preclinical studies and studies in patients were presented. Read More

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http://dx.doi.org/10.1111/epi.14555DOI Listing
October 2018
9 Reads

Improved seizure control and regaining cognitive milestones after vagus nerve stimulation revision surgery in Lennox-Gastaut syndrome.

Epilepsy Behav Case Rep 2018 11;10:111-113. Epub 2018 Sep 11.

Department of Neurosurgery, Maastricht UMC +, P. Debyelaan 25, 6202 AZ Maastricht, the Netherlands.

We report a child with Lennox-Gastaut syndrome with an increase in seizure frequency and loss of psychomotor skills due to a disintegrated cervical VNS lead, not detected during standard device monitoring. The lead was completely removed and replaced by a new 303 lead on the same nerve segment. After reinitiating VNS, side effects forced us to switch it off, resulting in immediate seizure recurrence. Read More

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http://dx.doi.org/10.1016/j.ebcr.2018.08.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6197149PMC
September 2018
2 Reads

Pharmacokinetics of clobazam oral soluble film.

Epilepsia 2018 Nov 24;59(11):2153-2161. Epub 2018 Oct 24.

Aquestive Therapeutics, Inc, Warren, New Jersey.

Objective: Clobazam oral soluble film (COSF) is a novel dosage form under development for the adjunctive treatment of seizures associated with Lennox-Gastaut syndrome. The present study was undertaken to assess the pharmacokinetics of clobazam administered as single doses of COSF 20 and 10 mg compared with clobazam tablets (CTAB) 20 and 10 mg in healthy adults. A secondary objective was to assess the safety and tolerability of single doses of COSF 20 and 10 mg. Read More

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http://dx.doi.org/10.1111/epi.14581DOI Listing
November 2018
4 Reads

A phase I, randomized, open-label, single-dose, 3-period crossover study to evaluate the drug-drug interaction between ZX008 (fenfluramine HCl oral solution) and a regimen of stiripentol, clobazam, and valproate in healthy subjects
.

Int J Clin Pharmacol Ther 2019 Jan;57(1):11-19

Objective: Phase I, open-label, randomized, single-dose, 3-period crossover study assessing pharmacokinetics (PK) and safety of ZX008, a liquid oral formulation of fenfluramine (FFA) under development for adjunctive treatment of Dravet syndrome and Lennox-Gastaut syndrome, administered with and without a combined antiepileptic drug (AED) regimen of stiripentol (STP), valproate (VPA), and clobazam (CLB) (STP regimen).

Materials And Methods: 26 healthy adults were administered the following treatments: ZX008 0.8 mg/kg; STP 3,500 mg, CLB 20 mg, VPA 25 mg/kg (max. Read More

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http://dx.doi.org/10.5414/CP203276DOI Listing
January 2019
4 Reads

Evaluation of long-term safety, tolerability, and behavioral outcomes with adjunctive rufinamide in pediatric patients (≥1 to <4 years old) with Lennox-Gastaut syndrome: Final results from randomized study 303.

Eur J Paediatr Neurol 2018 Sep 27. Epub 2018 Sep 27.

Formerly of Eisai Neurology Business Group, Eisai Inc., 100 Tice Boulevard, Woodcliff Lake, NJ 07677, USA. Electronic address:

Objective: Evaluate the long-term safety, tolerability, and behavioral effects of adjunctive rufinamide in pediatric patients (≥1 to <4 years old) with inadequately controlled seizures associated with Lennox-Gastaut syndrome (LGS).

Methods: Study 303 (ClinicalTrials.gov identifier NCT01405053) was a multicenter, randomized, open-label, Phase III trial. Read More

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http://dx.doi.org/10.1016/j.ejpn.2018.09.010DOI Listing
September 2018
1.930 Impact Factor

Sulthiame add-on therapy in children with Lennox-Gastaut syndrome: A study of 44 patients.

Seizure 2018 Nov 27;62:55-58. Epub 2018 Sep 27.

Hospital de Pediatría A Fleming, Mendoza, Argentina.

Purpose: The aim of this study was to evaluate efficacy and tolerability of sulthiame as an add-on treatment in 44 patients with Lennox-Gastaut syndrome (LGS) refractory to other antiepileptic drugs and/or non-pharmacological treatment.

Methods: Patients were selected according to the following criteria: (1) age 4 years or older, (2) a diagnosis of LGS refractory to at least four previous antiepileptic drugs, alone or in combination. Neurologic examinations, brain magnetic resonance imaging, and repeated prolonged electroencephalography (EEG) or video-EEG studies were performed in all cases. Read More

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http://dx.doi.org/10.1016/j.seizure.2018.09.019DOI Listing
November 2018
10 Reads

Diagnosis switching and outcomes in a cohort of patients with potential epilepsy with myoclonic-atonic seizures.

Epilepsy Res 2018 Nov 24;147:95-101. Epub 2018 Sep 24.

Department of Pediatrics, Section of Neurology, Children's Hospital of Colorado, University of Colorado Anschutz Medical Campus, Aurora, CO, USA. Electronic address:

Introduction: There is overlap in the electroclinical features of many childhood epilepsy syndromes, especially those presenting with multiple seizure types, such as epilepsy with myoclonic-atonic seizures (EMAS) and Lennox-Gastaut syndrome (LGS). This study aimed to determine the frequency of diagnosis switching and the factors influencing epilepsy syndrome diagnosis in a cohort of children with possible EMAS, as well as to explore the relationship between epilepsy syndrome diagnoses, key electroclinical features, and clinically relevant outcomes.

Methods: This is a cross-sectional retrospective chart review of children treated at the Children's Hospital of Colorado with a potential diagnosis of EMAS. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09201211183040
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http://dx.doi.org/10.1016/j.eplepsyres.2018.09.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6223641PMC
November 2018
1 Read

A Comprehensive Overview of the Clinical Pharmacokinetics of Clobazam.

J Clin Pharmacol 2019 Jan 4;59(1):7-19. Epub 2018 Oct 4.

H. Lundbeck A/S, Valby, Denmark.

Clobazam (CLB) is a 1,5-benzodiazepine that has been widely used as an anxiolytic and antiseizure drug (ASD) since it was first synthesized over 50 years ago. CLB was approved in the United States in 2011 as adjunctive therapy for seizures in patients ≥2 years old with Lennox-Gastaut syndrome. CLB pharmacokinetics (PK) have been studied in single- and multiple-dose administrations in healthy subjects. Read More

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http://doi.wiley.com/10.1002/jcph.1313
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http://dx.doi.org/10.1002/jcph.1313DOI Listing
January 2019
1 Read

A Case of Lennox-Gastaut Syndrome in a 6-Year-Old Child with Moyamoya Disease.

J Neurosci Rural Pract 2018 Oct-Dec;9(4):658-659

Department of Neurology, Pt. B.D. Sharma Post Graduate Institute of Medical Sciences, Rohtak, Haryana, India.

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http://www.ruralneuropractice.com/text.asp?2018/9/4/658/2398
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http://dx.doi.org/10.4103/jnrp.jnrp_85_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6126310PMC
October 2018
2 Reads

Evolution and course of early life developmental encephalopathic epilepsies: Focus on Lennox-Gastaut syndrome.

Epilepsia 2018 Nov 26;59(11):2096-2105. Epub 2018 Sep 26.

Department of Pediatrics, Yale School of Medicine, New Haven, Connecticut.

Objectives: Developmental encephalopathic epilepsies (DEEs) are characterized by refractory seizures, disability, and early death. Opportunities to improve care and outcomes focus on West syndrome/infantile spasms (WS/IS). Lennox-Gastaut syndrome (LGS) is almost as common but receives little attention. Read More

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http://doi.wiley.com/10.1111/epi.14569
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http://dx.doi.org/10.1111/epi.14569DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6215498PMC
November 2018
1 Read

Burden of illness in patients with possible Lennox-Gastaut syndrome: A retrospective claims-based study.

Epilepsy Behav 2018 Nov 18;88:66-73. Epub 2018 Sep 18.

Greenwich Biosciences, Inc., Carlsbad, CA, United States of America.

Objective: Lennox-Gastaut syndrome (LGS) is a severe and treatment-resistant epilepsy syndrome characterized by multiple subtypes of intractable seizures, moderate to severe cognitive impairment, and slow spike-wave complexes on electroencephalographic (EEG) recordings. Lennox-Gastaut syndrome is also associated with increased risk for injury, reduced quality of life, long-term disability, and early mortality. By evaluating private and public US medical insurance claims, we quantified healthcare utilization and direct costs in patients with possible LGS. Read More

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http://dx.doi.org/10.1016/j.yebeh.2018.08.032DOI Listing
November 2018
1 Read

Vagal Nerve Stimulation: Indications for Revision in Adult Refractory Epilepsy.

World Neurosurg 2018 Dec 11;120:e1047-e1053. Epub 2018 Sep 11.

Allegheny General Hospital, Department of Neurosurgery, Pittsburgh, Pennsylvania, USA.

Objective: The goal of this study was to assess the indications of revision for vagal nerve stimulation at a single institution in an adult population with drug-resistant epilepsy.

Methods: This was a retrospective review of a prospectively collected database who underwent vagal nerve stimulator implantation for drug-resistant epilepsy during 1992-2017. Patients receiving vagal nerve stimulation (VNS) implants were monitored throughout their perioperative and postoperative course and were classified according to type of seizure at the time of diagnosis and indications for VNS revision. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S18788750183202
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http://dx.doi.org/10.1016/j.wneu.2018.09.008DOI Listing
December 2018
8 Reads

[Transional syndrome: from West to Lennox-Gastaut syndromes].

Medicina (B Aires) 2018 ;78 Suppl 2:2-5

Universidad Autónoma de Aguascalientes, México. E-mail:

West syndrome or infantile spasms is an epileptic encephalopathy, classified as generalized epilepsies and syndromes. There are multiple reports of the evolution from West to Lennox-Gastaut syndrome of 25 up to 60%, without a specific cause is determined. It has been reported that they may be only an epileptic entity age dependent that it would be in relation to the degree of brain immaturity. Read More

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January 2018
1 Read

Cannabis for the Treatment of Epilepsy: an Update.

Curr Neurol Neurosci Rep 2018 Sep 8;18(11):73. Epub 2018 Sep 8.

Department of Neurology, University of Alabama at Birmingham Epilepsy Center, Birmingham, AL, USA.

Purpose Of Review: For millennia, there has been interest in the use of cannabis for the treatment of epilepsy. However, it is only recently that appropriately powered controlled studies have been completed. In this review, we present an update on the research investigating the use of cannabidiol (CBD), a non-psychoactive component of cannabis, in the treatment of epilepsy. Read More

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http://link.springer.com/10.1007/s11910-018-0882-y
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http://dx.doi.org/10.1007/s11910-018-0882-yDOI Listing
September 2018
8 Reads

Long-term Outcome of Resective Epilepsy Surgery in Patients With Lennox-Gastaut Syndrome.

Pediatrics 2018 Oct 7;142(4). Epub 2018 Sep 7.

Epilepsy Research Institute, College of Medicine,

Objective: We aimed to evaluate the long-term outcome of resective epilepsy surgery in patients with Lennox-Gastaut syndrome (LGS).

Methods: We reviewed the case reports of 90 patients with LGS who had undergone resective surgery between 2003 and 2014 at the Severance Children's Hospital and managed them for a minimum period of 2 years.

Results: At the time of surgery, the patients were between 3. Read More

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http://pediatrics.aappublications.org/lookup/doi/10.1542/ped
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http://dx.doi.org/10.1542/peds.2018-0449DOI Listing
October 2018
17 Reads

Analysis of electroencephalogram-derived indexes for anesthetic depth monitoring in pediatric patients with intellectual disability undergoing dental surgery.

J Dent Anesth Pain Med 2018 Aug 28;18(4):235-244. Epub 2018 Aug 28.

Anesthesiology, Faculdade de Medicina da Universidade do Porto, Hospital de São João, Porto, Portugal.

Background: Patients with intellectual disability (ID) often require general anesthesia during oral procedures. Anesthetic depth monitoring in these patients can be difficult due to their already altered mental state prior to anesthesia. In this study, the utility of electroencephalographic indexes to reflect anesthetic depth was evaluated in pediatric patients with ID. Read More

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http://dx.doi.org/10.17245/jdapm.2018.18.4.235DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6115373PMC

Pharmaceutical Approval Update.

P T 2018 Sep;43(9):528-530

Baricitinib (Olumiant) tablets for rheumatoid arthritis; plazomicin (Zemdri) injection for complicated urinary tract infections; and cannabidiol (Epidiolex) oral solution for seizures with Lennox-Gastaut syndrome and Dravet syndrome. Read More

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6110637PMC
September 2018

Rufinamide efficacy and safety in children aged 1-4 years with Lennox-Gastaut syndrome.

Brain Dev 2018 Nov 27;40(10):897-903. Epub 2018 Aug 27.

Division of Pediatric Neurology, Pediatric Epilepsy Clinics, Severance Children's Hospital, Epilepsy Research Institute, Yonsei University College of Medicine, Seoul, Republic of Korea. Electronic address:

Purpose: The treatment options for Lennox-Gastaut syndrome (LGS), a pediatric epileptic syndrome, are limited, especially in younger children. Rufinamide tablets were safe and effective as an add-on treatment in Korean children and adolescents <20 years of age with LGS. This subgroup analysis aimed to evaluate the efficacy and safety of rufinamide tablets in LGS pediatric patients aged 1-4 years. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S03877604183038
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http://dx.doi.org/10.1016/j.braindev.2018.07.020DOI Listing
November 2018
9 Reads

A pilot, open-label study of the effectiveness and tolerability of low-dose ZX008 (fenfluramine HCl) in Lennox-Gastaut syndrome.

Epilepsia 2018 Oct 26;59(10):1881-1888. Epub 2018 Aug 26.

Division of Pediatric Neurology, Antwerp University Hospital, University of Antwerp, Edegem, Belgium.

Objective: Lennox-Gastaut syndrome (LGS) is a drug-resistant, childhood onset electroclinical epilepsy syndrome with multiple seizure types and diagnostic electroencephalogram findings. ZX008 (fenfluramine HCl oral solution) was well tolerated and reduced seizure frequency in Dravet syndrome, prompting this phase 2, open-label, dose-finding study of add-on ZX008 in patients with LGS (NCT02655198).

Methods: Eligible treatment-refractory patients with LGS aged 3-18 years with ≥4 documented convulsive seizures (CS) in the prior 4 weeks were administered adjunctive ZX008 twice daily at an initial dose of 0. Read More

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http://dx.doi.org/10.1111/epi.14540DOI Listing
October 2018

Cannabidiol in the Lennox-Gastaut Syndrome.

N Engl J Med 2018 08;379(8):794

National and Kapodistrian University of Athens, Athens, Greece

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http://www.nejm.org/doi/10.1056/NEJMc1807878
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http://dx.doi.org/10.1056/NEJMc1807878DOI Listing
August 2018
10 Reads

Cannabidiol in the Lennox-Gastaut Syndrome.

N Engl J Med 2018 08;379(8):794

University of Sydney, Sydney, NSW, Australia

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http://www.nejm.org/doi/10.1056/NEJMc1807878
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http://dx.doi.org/10.1056/NEJMc1807878DOI Listing
August 2018
10 Reads

Cannabidiol in the Lennox-Gastaut Syndrome.

N Engl J Med 2018 08;379(8):795

Greenwich Biosciences, Carlsbad, CA

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http://www.nejm.org/doi/10.1056/NEJMc1807878
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http://dx.doi.org/10.1056/NEJMc1807878DOI Listing
August 2018
9 Reads

Efficacy and Safety of Adjunctive Cannabidiol in Patients with Lennox-Gastaut Syndrome: A Systematic Review and Meta-Analysis.

CNS Drugs 2018 Oct;32(10):905-916

Neurological Clinic, Department of Experimental and Clinical Medicine, Marche Polytechnic University, Via Conca 71, 60020, Ancona, Italy.

Background: Lennox-Gastaut syndrome (LGS) is a severe developmental epileptic encephalopathy, and available interventions fail to control seizures in most patients. Cannabidiol (CBD) is a major chemical of marijuana, which has anti-seizure properties and different mechanisms of action compared with other approved antiepileptic drugs (AEDs).

Objective: The aim was to evaluate the efficacy and safety of CBD as adjunctive treatment for seizures in patients with LGS using meta-analytical techniques. Read More

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http://dx.doi.org/10.1007/s40263-018-0558-9DOI Listing
October 2018
11 Reads

Lacosamide-induced excessive laughing in a patient with Lennox-Gastaut syndrome.

Epilepsy Behav Case Rep 2018 6;10:1-3. Epub 2018 Feb 6.

King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia.

Lacosamide is one of the third-generation antiseizure drugs that block voltage-gated sodium channels by enhancing slow inactivation. The most common adverse effects of lacosamide include dizziness, headache, nausea, vomiting, diplopia, fatigue, and sedation. Less common side effects include memory impairment, weight gain, rash, and atrioventricular block. Read More

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http://dx.doi.org/10.1016/j.ebcr.2018.01.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6061898PMC
February 2018
7 Reads

Multifocal epilepsy in children is associated with increased long-distance functional connectivity: An explorative EEG-fMRI study.

Eur J Paediatr Neurol 2018 Jul 5. Epub 2018 Jul 5.

Department of Neuropediatrics, Christian-Albrechts-University, Kiel, Germany.

Objective: Multifocal epileptic activity is an unfavourable feature of a number of epileptic syndromes (Lennox-Gastaut syndrome, West syndrome, severe focal epilepsies) which suggests an overall vulnerability of the brain to pathological synchronization. However, the mechanisms of multifocal activity are insufficiently understood. This explorative study investigates whether pathological connectivity within brain areas of the default mode network as well as thalamus, brainstem and retrosplenial cortex may predispose individuals to multifocal epileptic activity. Read More

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http://dx.doi.org/10.1016/j.ejpn.2018.07.001DOI Listing
July 2018
1 Read

Interstitial Stereotactic Laser Anterior Corpus Callosotomy: A Report of 2 Cases with Operative Technique and Effectiveness.

Neurosurgery 2018 Jul 5. Epub 2018 Jul 5.

Department of Neurosurgery, The University of Chicago, Chicago, Illinois.

Background And Importance: Corpus callosotomy is an effective palliative treatment for medically intractable Lennox-Gastaut syndrome (LGS) that disrupts the interhemispheric synchronization of epileptiform discharges. However, traditional open corpus callosotomy carries a significant risk of surgical complications associated with craniotomy and a parafalcine approach to the corpus callosum. Here, we report 2 cases of anterior corpus callosotomy using MRI-guided stereotactic laser interstitial thermal therapy (LITT) as a minimally invasive technique for mitigating the risks of craniotomy while achieving favorable outcomes. Read More

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http://dx.doi.org/10.1093/neuros/nyy273DOI Listing
July 2018
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Brain cooling reduces the risk of post-neonatal epilepsy in newborns affected by moderate to severe hypoxic-ischemic encephalopathy.

Minerva Pediatr 2018 Jul 2. Epub 2018 Jul 2.

Division of Neonatal Intensive Care Unit, Department of Pediatrics, University Hospital, Modena, Italy.

Background: Neonatal hypoxic-ischemic encephalopathy is still a significant cause of neonatal death and neurodevelopmental disabilities, such as cerebral palsy, mental delay, and epilepsy. After the introduction of therapeutic hypothermia, the prognosis of hypoxic-ischemic encephalopathy has improved, with reduction of death and disabilities. However, few studies evaluated whether hypothermia affects rate and severity of post-neonatal epilepsy. Read More

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http://dx.doi.org/10.23736/S0026-4946.18.05224-6DOI Listing
July 2018
2 Reads

Effect of Rufinamide on the kainic acid-induced excitotoxic neuronal death in the mouse hippocampus.

Arch Pharm Res 2018 Jul 8;41(7):776-783. Epub 2018 Jun 8.

Department of Pharmacy, College of Pharmacy, Dankook University, Cheonan, 31116, Republic of Korea.

Rufinamide (RUF) is a structurally unique anti-epileptic drug, used in the treatment of seizure disorders such as Lennox-Gastaut syndrome. In the present study, we investigated whether RUF protected against excitotoxic neuronal damage in the mouse hippocampal CA3 region after intraperitoneal kainic acid (KA) injection. Treatment with 25, 50 and 100 mg/kg RUF significantly decreased the KA-induced neuronal death in the hippocampal CA3 region in a dose-dependent manner. Read More

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http://dx.doi.org/10.1007/s12272-018-1043-1DOI Listing
July 2018
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The Lack of Effect of Food on the Pharmacokinetics of ZX008 (Fenfluramine Oral Solution): Results of a Single-dose, Two-period Crossover Study.

Clin Ther 2018 Aug 23;40(8):1338-1346. Epub 2018 Jun 23.

Zogenix, Inc., Emeryville, California. Electronic address:

Purpose: Fenfluramine is being developed as a low-dose adjunctive treatment for seizures in patients with Dravet syndrome and other epileptic encephalopathies, including Lennox-Gastaut syndrome. Most patients with Dravet syndrome receive multiple antiepileptic drugs, making it challenging for caregivers to track correct administration times. The present Phase I study was conducted to determine the effect of food on the pharmacokinetic properties of fenfluramine. Read More

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http://dx.doi.org/10.1016/j.clinthera.2018.05.013DOI Listing
August 2018
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Investigation of neuronal auto-antibodies in children diagnosed with epileptic encephalopathy of unknown cause.

Brain Dev 2018 Nov 21;40(10):909-917. Epub 2018 Jun 21.

Istanbul University, Institute of Experimental Medical Research, Department of Neuroscience, Istanbul, Turkey.

Aim: Cryptogenic forms of epileptic encephalopathies (EE) with their well-known features of drug-resistance, mental deterioration and partial response to immunotherapies are ideal candidates for screening for neuronal autoantibodies (NAA).

Method: Fifty consecutive pediatric patients with a diagnosis of EE of unknown cause were included. Nine NAAs were tested by ELISA, RIA or cell-based assays. Read More

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http://dx.doi.org/10.1016/j.braindev.2018.06.002DOI Listing
November 2018
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Ictal signs in tuberous sclerosis complex: Clinical and video-EEG features in a large series of recorded seizures.

Epilepsy Behav 2018 Aug 12;85:14-20. Epub 2018 Jun 12.

Epilepsy Center-Child Neuropsychiatric Unit, ASST Santi Paolo e Carlo, Milan, Italy; Department of Health Sciences, University of Milan, Italy.

Epilepsy is the most common neurological symptom in tuberous sclerosis complex (TSC), occurring in 72-85% of affected individuals. Despite the large number of patients reported, their electroclinical phenotype has been rarely described. We analyzed seizure semiology through ictal video-electroencephalography (V-EEG) recordings in a large series of patients. Read More

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http://dx.doi.org/10.1016/j.yebeh.2018.05.027DOI Listing
August 2018
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Practice guideline update summary: Efficacy and tolerability of the new antiepileptic drugs II: Treatment-resistant epilepsy: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology and the American Epilepsy Society.

Neurology 2018 Jul 13;91(2):82-90. Epub 2018 Jun 13.

From Miller School of Medicine (A.M.K.), University of Miami, FL; Bronson Methodist Hospital (E.A.), Kalamazoo, MI; Charleston Area Medical Center (D.G.), Charleston, WV; Mount Sinai Beth Israel (C.H.), New York, NY; Children's Hospital, Harvard Medical School (B.B.), Boston, MA; Cleveland Clinic Foundation (J.F.B.), OH; Department of Neurology (B.-A.K.), School of Medicine, Nashville, TN; Cooper Medical School (E.B.-D.), Rowan University, Cherry Hill, NJ; Alexian Brothers Medical Group (E.L.P.), Hoffman Estates, IL; School of Medicine (J.S.), University of California in Los Angeles; University of Vermont Medical Center (D.H.), Burlington; Children's Hospital (M.N.), University of California San Diego School of Medicine; School of Pharmacy (B.G.), University of Wisconsin, Madison; Emory University School of Medicine (E.F.), Atlanta, GA; and New York University (J.F.), New York.

Objective: To update the 2004 American Academy of Neurology guideline for managing treatment-resistant (TR) epilepsy with second- and third-generation antiepileptic drugs (AEDs).

Methods: 2004 criteria were used to systemically review literature (January 2003 to November 2015), classify pertinent studies according to the therapeutic rating scheme, and link recommendations to evidence strength.

Results: Forty-two articles were included. Read More

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http://dx.doi.org/10.1212/WNL.0000000000005756DOI Listing
July 2018
14 Reads

Investigational cannabinoids in seizure disorders, what have we learned thus far?

Expert Opin Investig Drugs 2018 Jun 6;27(6):535-541. Epub 2018 Jun 6.

a Faculty of Medical Sciences , University of Kragujevac , Kragujevac , Serbia.

Introduction: The anticonvulsant activity of cannabinoids attracted much attention in the last decade. Cannabinoids that are currently investigated with the intention of making them drugs for the treatment of epilepsy are cannabidiol, cannabidivarin, Δ9-tetrahydrocannabivarin, and Δ9-tetrahydrocannabinolic acid. Areas covered:In this review, the authors look at the results of preclinical and clinical studies with investigational cannabinoids. Read More

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http://dx.doi.org/10.1080/13543784.2018.1482275DOI Listing
June 2018
7 Reads

Association of GABAA Receptor Gene with Epilepsy Syndromes.

J Mol Neurosci 2018 Jun 21;65(2):141-153. Epub 2018 May 21.

Maulana Azad Medical College and Associated Hospitals, Bahadur Shah Zafar Marg, New Delhi, 110002, India.

GABA has always been an inviting target in the etiology and treatment of epilepsy. The GABRA1, GABRG2, and GABRD genes provide instructions for making α1, ϒ2, and δ subunits of GABAA receptor protein respectively. GABAA is considered as one of the most important proteins and has found to play an important role in many neurological disorders. Read More

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http://dx.doi.org/10.1007/s12031-018-1081-7DOI Listing
June 2018
5 Reads
2.340 Impact Factor

Review of the neurological benefits of phytocannabinoids.

Surg Neurol Int 2018 26;9:91. Epub 2018 Apr 26.

Department of Neurosurgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.

Background: Numerous physical, psychological, and emotional benefits have been attributed to marijuana since its first reported use in 2,600 BC in a Chinese pharmacopoeia. The phytocannabinoids, cannabidiol (CBD), and delta-9-tetrahydrocannabinol (Δ9-THC) are the most studied extracts from subspecies hemp and marijuana. CBD and Δ9-THC interact uniquely with the endocannabinoid system (ECS). Read More

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http://dx.doi.org/10.4103/sni.sni_45_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5938896PMC
April 2018
3 Reads

Effect of Cannabidiol on Drop Seizures in the Lennox-Gastaut Syndrome.

N Engl J Med 2018 May;378(20):1888-1897

From New York University Langone Comprehensive Epilepsy Center, New York (O.D.); Nationwide Children's Hospital and the Ohio State University College of Medicine, Columbus (A.D.P.), and the University of Cincinnati Medical Center, Department of Neurology, Cincinnati (M.P.) - all in Ohio; UCL Great Ormond Street Institute of Child Health, London (J.H.C.), GW Research Ltd., Cambridge (S.M.G., C.R., D.C.), and the Royal Hospital for Children and School of Medicine, University of Glasgow, Glasgow (S.M.Z.) - all in the United Kingdom; Refractory Epilepsy Unit, Neurology Service, Hospital Universitario y Politécnico La Fe, Valencia, Spain (V.V.); the Divisions of Child and Adolescent Neurology and Epilepsy, Department of Neurology, Mayo Clinic, Rochester, MN (E.C.W.); and Greenwich Biosciences, Carlsbad, CA (K.E.V.).

Background: Cannabidiol has been used for treatment-resistant seizures in patients with severe early-onset epilepsy. We investigated the efficacy and safety of cannabidiol added to a regimen of conventional antiepileptic medication to treat drop seizures in patients with the Lennox-Gastaut syndrome, a severe developmental epileptic encephalopathy.

Methods: In this double-blind, placebo-controlled trial conducted at 30 clinical centers, we randomly assigned patients with the Lennox-Gastaut syndrome (age range, 2 to 55 years) who had had two or more drop seizures per week during a 28-day baseline period to receive cannabidiol oral solution at a dose of either 20 mg per kilogram of body weight (20-mg cannabidiol group) or 10 mg per kilogram (10-mg cannabidiol group) or matching placebo, administered in two equally divided doses daily for 14 weeks. Read More

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http://dx.doi.org/10.1056/NEJMoa1714631DOI Listing
May 2018
4 Reads