1,146 results match your criteria EEG in Common Epilepsy Syndromes


Phenotypic Spectrum of Seizure Disorders in MBD5-Associated Neurodevelopmental Disorder.

Neurol Genet 2021 Apr 18;7(2):e579. Epub 2021 Mar 18.

Research Institute of the McGill University Health Centre (K.M.), Montreal, PQ; Division of Child Neurology (K.M.), Department of Pediatrics, Montreal Children's Hospital, McGill University, Montreal, PQ; Department of Neurology & Neurosurgery (K.M.), Montreal Children's Hospital, McGill University, Montreal, PQ; Child Neurology and Psychiatry (C.M.), Salesi Pediatric Hospital, United Hospitals of Ancona, Ancona, Italy; Division of Genetic Medicine (G.L.C., J.N., H.C.M.), Department of Pediatrics, University of Washington, Seattle, WA; Department of Neurology (A.M.), Great Ormond Street Hospital for Children, London, UK; Developmental Neurosciences Programme (A.M.), UCL Great Ormond Street Institute of Child Health, London, UK; Neurology Network Melbourne (J.P.), Melbourne, Victoria, Australia; Murdoch Children's Research Institute (C.S., I.E.S.), Parkville, Victoria, Australia; Department of Paediatrics and Child Health (T.S.), School of Medicine and Health Sciences, University of Otago, Wellington, New Zealand; Division of Neurology (S.M.), Department of Pediatrics, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, Manitoba, Canada; Neurology Unit and Neurogenetic Laboratories (C.B., A.R., R.G.), Meyer Children's Hospital, Florence, Italy; Department of Clinical Genetics (R.H.S.), Great Ormond Street Hospital, London, UK; Epilepsy Research Centre (I.E.S.), Department of Medicine, The University of Melbourne, Austin Health, Heidelberg, Victoria, Australia; Department of Paediatrics (I.E.S.), Royal Children's Hospital, The University of Melbourne, Parkville, Victoria, Australia; and The Florey Institute of Neuroscience and Mental Health (I.E.S.), Heidelberg, Victoria, Australia.

Objective: To describe the phenotypic spectrum in patients with MBD5-associated neurodevelopmental disorder (MAND) and seizures; features of MAND include intellectual disability, epilepsy, psychiatric features of aggression and hyperactivity, and dysmorphic features including short stature and microcephaly, sleep disturbance, and ataxia.

Methods: We performed phenotyping on patients with deletions, duplications, or point mutations and a history of seizures.

Results: Twenty-three patients with MAND and seizures were included. Read More

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Intravenous immunoglobulin response in new-onset refractory status epilepticus (NORSE) COVID-19 adult patients.

J Neurol 2021 Mar 11. Epub 2021 Mar 11.

Clinical Unit of Neurology, Department of Medicine, Surgery and Health Sciences, Trieste University Hospital-ASUGI, University of Trieste, Strada di Fiume, 447-34149, Trieste, Italy.

Neurological manifestations may be common in COVID-19 patients. They may include several syndromes, such as a suggested autoimmune abnormal response, which may result in encephalitis and new-onset refractory status epilepticus (NORSE). Quickly recognizing such cases and starting the most appropriate therapy is mandatory due to the related rapid worsening and bad outcomes. Read More

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Ambulatory EEG to Classify the Epilepsy Syndrome.

J Clin Neurophysiol 2021 Mar;38(2):87-91

Department of Neurology, Northwestern University, Chicago, Illinois, U.S.A.

Summary: Recording of interictal epileptiform discharges to classify the epilepsy syndrome is one of the most common indications for ambulatory EEG. Ambulatory EEG has superior sampling compared with standard EEG recordings and advantages in terms of cost-effectiveness and convenience compared with a prolonged inpatient EEG study. Ambulatory EEG allows for EEG recording in all sleep stages and transitional states, which can be very helpful in capturing interictal epileptiform discharges. Read More

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The severe epilepsy syndromes of infancy: A population-based study.

Epilepsia 2021 02 21;62(2):358-370. Epub 2021 Jan 21.

Department of Neurology, Royal Children's Hospital, Melbourne, Vic, Australia.

Objective: To study the epilepsy syndromes among the severe epilepsies of infancy and assess their incidence, etiologies, and outcomes.

Methods: A population-based cohort study was undertaken of severe epilepsies with onset before age 18 months in Victoria, Australia. Two epileptologists reviewed clinical features, seizure videos, and electroencephalograms to diagnose International League Against Epilepsy epilepsy syndromes. Read More

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February 2021

Auras in intractable frontal lobe epilepsy: Clinical characteristics, values, and limitations.

Epilepsy Behav 2021 02 8;115:107724. Epub 2021 Jan 8.

Beijing Institute of Functional Neurosurgery, Xuanwu Hospital, Capital Medical University, No. 45, Changchun Street, Xicheng District, Beijing 100053, China; Department of Functional Neurology, Xuanwu Hospital, Capital Medical University, No. 45, Changchun Street, Xicheng District, Beijing 100053, China. Electronic address:

Auras are essential in preoperative evaluation and can provide valuable information for delineating seizure onset zones. Frontal lobe epilepsy (FLE) is the second most common focal epilepsy, while a few studies have focused on auras in FLE. To better understand FLE, we analyzed the clinical characteristics, values, and limitations of auras in FLE. Read More

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February 2021

The phenotypic spectrum of X-linked, infantile onset ALG13-related developmental and epileptic encephalopathy.

Epilepsia 2021 02 7;62(2):325-334. Epub 2021 Jan 7.

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

Objective: Asparagine-linked glycosylation 13 (ALG13) deficiencies have been repeatedly described in the literature with the clinical phenotype of a developmental and epileptic encephalopathy (DEE). Most cases were females carrying the recurrent ALG13 de novo variant, p.(Asn107Ser), with normal transferrin electrophoresis. Read More

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February 2021

Persistent treatment resistance in genetic generalized epilepsy: A long-term outcome study in a tertiary epilepsy center.

Epilepsia 2020 11 14;61(11):2452-2460. Epub 2020 Oct 14.

Epilepsy Unit, Department of Human Neurosciences, Policlinico "Umberto I", "Sapienza" University, Rome, Italy.

Objective: To assess prognostic patterns and investigate clinical and electroencephalography (EEG) variables associated with persistent treatment resistance in a population of genetic generalized epilepsy (GGE) patients with a long-term follow-up.

Methods: Data from GGE patients followed from 1975 to 2019 were reviewed retrospectively. Subjects with a follow-up >10 years, starting from epilepsy diagnosis, were included. Read More

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November 2020

A study of medial and lateral temporal lobe epilepsy based on stereoelectroencephalography.

Chin Med J (Engl) 2020 Dec 2;134(1):68-72. Epub 2020 Dec 2.

Beijing Institute of Functional Neurosurgery, Xuanwu Hospital, Capital Medical University, Beijing 100053, China.

Background: Patients with temporal lobe epilepsy (TLE) originating from different seizure onset zones had distinct electrophysiological characteristics and surgical outcomes. In this study, we aimed to investigate the relationship between the origin and prognosis of TLE, and the stereoelectroencephalography (SEEG) features.

Methods: Thirty patients with TLE, who underwent surgical treatment in our functional neurosurgery department from January 2016 to December 2017, were enrolled in this study. Read More

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December 2020

A needs assessment of pediatric epilepsy surgery in Haiti.

J Neurosurg Pediatr 2020 Nov 27:1-7. Epub 2020 Nov 27.

3Division of Neurosurgery, Department of Surgery, University of Toronto, Ontario.

Objective: Epilepsy disproportionately affects low- and/or middle-income countries (LMICs). Surgical treatments for epilepsy are potentially curative and cost-effective and may improve quality of life and reduce social stigmas. In the current study, the authors estimate the potential need for a surgical epilepsy program in Haiti by applying contemporary epilepsy surgery referral guidelines to a population of children assessed at the Clinique d'Épilepsie de Port-au-Prince (CLIDEP). Read More

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November 2020

Management of Lennox-Gastaut syndrome beyond childhood: A comprehensive review.

Authors:
Debopam Samanta

Epilepsy Behav 2021 01 24;114(Pt A):107612. Epub 2020 Nov 24.

Child Neurology Section, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States. Electronic address:

Lennox-Gastaut syndrome (LGS) is a childhood-onset epileptic encephalopathy characterized by multiple types of medically intractable seizures, cognitive impairment, and generalized slow spike-wave discharges in electroencephalography (EEG). Although the onset of this epileptic syndrome occurs typically before eight years of age with a peak age between 3 and 5 years, lifelong persistence of the syndrome is usual. The evolution of clinical features, EEG findings, and paucity of knowledge about LGS among adult health care providers can make LGS significantly underdiagnosed in the adult population. Read More

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January 2021

Connectome biomarkers of drug-resistant epilepsy.

Epilepsia 2021 01 25;62(1):6-24. Epub 2020 Nov 25.

Multimodal Imaging and Connectome Analysis Laboratory, Montreal Neurological Institute and Hospital, McGill University, Montreal, Quebec, Canada.

Drug-resistant epilepsy (DRE) considerably affects patient health, cognition, and well-being, and disproportionally contributes to the overall burden of epilepsy. The most common DRE syndromes are temporal lobe epilepsy related to mesiotemporal sclerosis and extratemporal epilepsy related to cortical malformations. Both syndromes have been traditionally considered as "focal," and most patients benefit from brain surgery for long-term seizure control. Read More

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January 2021

Epilepsy with myoclonic-atonic seizures (Doose syndrome): Clarification of diagnosis and treatment options through a large retrospective multicenter cohort.

Epilepsia 2021 01 14;62(1):120-127. Epub 2020 Nov 14.

Children's Hospital of Colorado, University of Colorado Denver Anschutz School of Medicine, Aurora, CO, USA.

Objective: Epilepsy with myoclonic-atonic seizures (EMAS) is a rare childhood onset epileptic encephalopathy. There is no clear consensus for recommended treatments, and pharmacoresistance is common. To better assess the clinical phenotype, most effective treatment, and determinants of cognitive and seizure outcomes, three major pediatric epilepsy centers combined data, creating the largest cohort of patients with EMAS ever studied to date. Read More

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January 2021

An Update to Approach to the Childhood Electroclinical Syndromes.

Indian J Pediatr 2020 Dec 5;87(12):1029-1039. Epub 2020 Nov 5.

Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Childhood Electroclinical Syndrome (CES) is a term which refers to distinct epilepsies of childhood which have peculiar similarities such as the age of onset, seizure semiology, EEG and prognosis. With advancements in the diagnostics and genetics, pediatric epilepsy is entering in the age of precision medicine. The present paper provides an update of CES in light of recent advances in the terminologies and classifications by International League Against Epilepsy and genetic underpinnings of epilepsy. Read More

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December 2020

Progressive Myoclonus Epilepsy Caused by a Homozygous Splicing Variant of SLC7A6OS.

Ann Neurol 2021 02 5;89(2):402-407. Epub 2020 Nov 5.

Genetics Department, Lyon Civil Hospices, Lyon, France.

Exome sequencing was performed in 2 unrelated families with progressive myoclonus epilepsy. Affected individuals from both families shared a rare, homozygous c.191A > G variant affecting a splice site in SLC7A6OS. Read More

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February 2021

Oral language in children with benign childhood epilepsy with centrotemporal spikes.

Epilepsy Behav 2020 10 9;111:107328. Epub 2020 Sep 9.

Departamento de Pediatria do Hospital Beatriz Ângelo, Portugal.

Purpose: Benign childhood epilepsy with centrotemporal spikes is one of the most common childhood disorders. Despite the benignity usually attributed to this epileptic syndrome, several studies have demonstrated that these children have cognitive disabilities. Among these disturbances, language disorders have been the less studied in depth. Read More

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October 2020

Standard procedures for the diagnostic pathway of sleep-related epilepsies and comorbid sleep disorders: an EAN, ESRS and ILAE-Europe consensus review.

Eur J Neurol 2021 Jan 21;28(1):15-32. Epub 2020 Sep 21.

IRCCS Istituto delle Scienze Neurologiche di Bologna, Ospedale Bellaria, Bologna, Italy.

Background And Purpose: Some epilepsy syndromes (sleep-related epilepsies, SREs) have a strong link with sleep. Comorbid sleep disorders are common in patients with SRE and can exert a negative impact on seizure control and quality of life. Our purpose was to define the standard procedures for the diagnostic pathway of patients with possible SRE (scenario 1) and the general management of patients with SRE and comorbidity with sleep disorders (scenario 2). Read More

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January 2021

Persistent abnormalities in Rolandic thalamocortical white matter circuits in childhood epilepsy with centrotemporal spikes.

Epilepsia 2020 11 18;61(11):2500-2508. Epub 2020 Sep 18.

Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, USA.

Objective: Childhood epilepsy with centrotemporal spikes (CECTS) is a common, focal, transient, developmental epilepsy syndrome characterized by unilateral or bilateral, independent epileptiform spikes in the Rolandic regions of unknown etiology. Given that CECTS presents during a period of dramatic white matter maturation and thatspikes in CECTS are activated during non-rapid eye movement (REM) sleep, we hypothesized that children with CECTS would have aberrant development of white matter connectivity between the thalamus and the Rolandic cortex. We further tested whether Rolandic thalamocortical structural connectivity correlates with spike rate during non-REM sleep. Read More

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November 2020

Baseline neuropsychological characteristics in patients with epilepsy with left temporal lobe encephaloceles compared with left mesial temporal sclerosis.

Epilepsy Behav 2020 11 9;112:107397. Epub 2020 Sep 9.

Department of Neurology, Vanderbilt University Medical Center, A-0118 Medical Center North, 1161 21st Avenue South, Nashville, TN 37232, United States. Electronic address:

Background: Temporal lobe encephaloceles (TE) are increasingly recognized as a cause of drug-resistant temporal lobe epilepsy. Improved recognition of these lesions offers an opportunity to treat them with a limited resection sparing the hippocampus. However, as they can be difficult to identify on imaging, additional clues pointing to the diagnosis can be helpful. Read More

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November 2020

ADHD and ADHD-related neural networks in benign epilepsy with centrotemporal spikes: A systematic review.

Epilepsy Behav 2020 11 21;112:107448. Epub 2020 Sep 21.

Department of Human Neuroscience and Mental Health, Child Neurology and Psychiatry, Policlinico Umberto I, Rome, Italy. Electronic address:

Background: Attention-deficit/hyperactivity disorder (ADHD) and benign epilepsy with centrotemporal spikes (BECTS or rolandic epilepsy) present with a very high level of comorbidity. We aimed to review the existing literature focusing on two aspects: the possible role of epileptic activity in the damage of ADHD-related neural networks and the clinical approach to patients presenting with both conditions.

Material And Methods: A systematic review was performed using Sapienza Library System and PubMed. Read More

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November 2020

Use of the International League Against Epilepsy (ILAE) 1989, 2010, and 2017 Classification of Epilepsy in children in a low-resource setting: A hospital-based cross-sectional study.

Epilepsia Open 2020 Sep 21;5(3):397-405. Epub 2020 Jun 21.

Department of Pediatrics Lady Hardinge Medical College and associated Kalawati Saran Children's Hospital New Delhi India.

Objectives: This cross-sectional study was designed to test the applicability of the 1989, 2010, and 2017 International League Against Epilepsy (ILAE) classification of epilepsy in children from a resource-limited setting in India.

Methods: Classification of seizure types and syndromes was done through parental interviews and review of medical records in children with epilepsy aged one month to 18 years. Available investigations including EEG, MRI, and metabolic/genetic tests were used in classifying patients as per the 1989, 2010, and 2017 ILAE (level II-epilepsy type) classification. Read More

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September 2020

Prefrontal seizure classification based on stereo-EEG quantification and automatic clustering.

Epilepsy Behav 2020 11 6;112:107436. Epub 2020 Sep 6.

Aix Marseille Univ, INSERM, INS, Inst Neurosci Syst, Marseille, France; APHM, Timone Hospital, Epileptology Department, Marseille, France. Electronic address:

Purpose: Frontal seizures are organized according to anatomo-functional subdivisions of the frontal lobe. Prefrontal seizures have been the subject of few detailed studies to date. The objective of this study was to identify subcategories of prefrontal seizures based on seizure onset quantification and to look for semiological differences. Read More

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November 2020

[Pediatric epilepsies].

Rev Prat 2020 Apr;70(4):421-426

Service de neurologie pédiatrique, hôpital universitaire Robert-Debré, Paris, France.

Pediatric epilepsies. Epilepsies are a heterogeneous group of diseases characterized by the recurrence of epileptic seizures. The incidence is higher in children than in adults. Read More

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PIK3R2/Pik3r2 Activating Mutations Result in Brain Overgrowth and EEG Changes.

Ann Neurol 2020 12 28;88(6):1077-1094. Epub 2020 Sep 28.

Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.

Objective: Mutations in phosphatidylinositol-4,5-bisphosphate 3-kinase (PI3K) complex have been associated with a broad spectrum of brain and organ overgrowth syndromes. For example, mutations in phosphatidylinositol-3-kinase regulatory subunit 2 (PIK3R2) have been identified in human patients with megalencephaly polymicrogyria polydactyly hydrocephalus (MPPH) syndrome, which includes brain overgrowth. To better understand the pathogenesis of PIK3R2-related mutations, we have developed and characterized a murine model. Read More

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December 2020

[Clinical, structural and functional features of paroxismal syndrome in insular and temporal lobe tumors].

Zh Vopr Neirokhir Im N N Burdenko 2020 ;84(4):36-45

Almazov National Medical Research Center, St. Petersburg, Russia.

Objective: To analyze the characteristics of paroxysmal syndrome in insular and temporal lobe tumors, to determine their relationship with the histological structure of tumor, to assess the effect of tumor growth nature on severity of disease.

Material And Methods: A retrospective analysis enrolled 80 patients aged 11 - 80 years with insular and temporal lobe tumors and symptomatic epilepsy. All patients underwent surgery at the Polenov National Research Neurosurgery Center in Almazov National Medical Research Center for the period from 2012 to 2018. Read More

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September 2020

Nonseizure consequences of Dravet syndrome, KCNQ2-DEE, KCNB1-DEE, Lennox-Gastaut syndrome, ESES: A functional framework.

Epilepsy Behav 2020 10 3;111:107287. Epub 2020 Aug 3.

CLIRINX, Dublin, Ireland.

Rationale: Developmental epilepsies and encephalopathies (DEEs) are characterized by many severe developmental impairments, which are not well-described. A functional framework could facilitate understanding of their nature and severity and guide the selection instruments to measure improvements in therapeutic trials.

Methods: An online survey administered through several parent-organized foundations utilized accepted functional classifications and questionnaires derived from common instruments to determine levels of mobility, fine motor, communication, and feeding functions. Read More

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October 2020

Trait impulsivity correlates with active myoclonic seizures in genetic generalized epilepsy.

Epilepsy Behav 2020 11 31;112:107260. Epub 2020 Jul 31.

Department of Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology & Neuroscience, King's College London, London, United Kingdom; MRC Centre for Neurodevelopmental Disorders, King's College London, London, United Kingdom; King's College Hospital, London, United Kingdom; Evelina London Children's Hospital, London, United Kingdom.

Background: Juvenile myoclonic epilepsy (JME) is a common subtype of genetic generalized epilepsy (GGE) arising in adolescence and is often associated with executive function (EF) deficits. Some EF components like response inhibition have been extensively evaluated in JME, but few studies have focused upon trait impulsivity or compared between GGE subtypes. The aim of the present study was to compare the association of trait impulsivity in JME with other GGE subtypes. Read More

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November 2020

Neuroligin 2 regulates absence seizures and behavioral arrests through GABAergic transmission within the thalamocortical circuitry.

Nat Commun 2020 07 27;11(1):3744. Epub 2020 Jul 27.

Neuroscience & Mental Health, The Hospital for Sick Children, Toronto, ON, M5G 1X8, Canada.

Epilepsy and autism spectrum disorders (ASD) are two distinct brain disorders but have a high rate of co-occurrence, suggesting shared pathogenic mechanisms. Neuroligins are cell adhesion molecules important in synaptic function and ASD, but their role in epilepsy remains unknown. In this study, we show that Neuroligin 2 (NLG2) knockout mice exhibit abnormal spike and wave discharges (SWDs) and behavioral arrests characteristic of absence seizures. Read More

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Absence Seizures in Children: Usual and the Unusual.

Authors:
Puneet Jain

Indian J Pediatr 2020 Dec 23;87(12):1047-1056. Epub 2020 Jul 23.

Epilepsy Program, Division of Neurology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, M5G1X8, Canada.

Absence seizures are commonly encountered in clinical practice. The diagnosis is usually straightforward in majority of cases. However, it may be challenging in patients with some atypical clinical or EEG features or less common epilepsy syndromes. Read More

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December 2020

Phenotypic spectrum of patients with GABRB2 variants: from mild febrile seizures to severe epileptic encephalopathy.

Dev Med Child Neurol 2020 10 20;62(10):1213-1220. Epub 2020 Jul 20.

Department of Pediatrics, Peking University First Hospital, Beijing, China.

Aim: To characterize the different phenotypes of GABRB2-related epilepsy and to establish a genotype-phenotype correlation.

Method: We used next-generation sequencing to identify GABRB2 variants in 15 patients.

Results: Eleven GABRB2 variants were novel and 12 were de novo. Read More

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October 2020

Targeted delivery of lacosamide-conjugated gold nanoparticles into the brain in temporal lobe epilepsy in rats.

Life Sci 2020 Sep 11;257:118081. Epub 2020 Jul 11.

Department of Physiology, School of Medicine, Koç University, Istanbul, Turkey. Electronic address:

Temporal lobe epilepsy (TLE) is the most common form of epilepsy with focal seizures, and currently available drugs may fail to provide a thorough treatment of the patients. The present study demonstrates the utility of glucose-coated gold nanoparticles (GNPs) as selective carriers of an antiepileptic drug, lacosamide (LCM), in developing a strategy to cross the blood-brain barrier to overcome drug resistance. Intravenous administration of LCM-loaded GNPs to epileptic animals yielded significantly higher nanoparticle levels in the hippocampus compared to the nanoparticle administration to intact animals. Read More

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September 2020