5,720 results match your criteria Epileptic and Epileptiform Encephalopathies


Dual Role for Astroglial Copper-Assisted Polyamine Metabolism during Intense Network Activity.

Biomolecules 2021 Apr 19;11(4). Epub 2021 Apr 19.

Functional Pharmacology Research Group, Research Centre for Natural Sciences, Institute of Organic Chemistry, H-1117 Budapest, Hungary.

Astrocytes serve essential roles in human brain function and diseases. Growing evidence indicates that astrocytes are central players of the feedback modulation of excitatory Glu signalling during epileptiform activity via Glu-GABA exchange. The underlying mechanism results in the increase of tonic inhibition by reverse operation of the astroglial GABA transporter, induced by Glu-Na symport. Read More

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A characteristic occipital epileptiform EEG pattern in ADCK3-related mitochondrial disease.

Epileptic Disord 2021 Apr 21. Epub 2021 Apr 21.

Department of Neurology and Clinical Neurophysiology, St. Olavs Hospital, Trondheim University Hospital, Norway, Department of Neuromedicine and Movement Science, Norwegian University of Science and Technology (NTNU), Trondheim, Norway.

Objective: ADCK3-related disease is a mitochondrial disorder associated with an abnormality of coenzyme Q metabolism. Ataxia and epilepsy are common, and the phenotype overlaps with other mitochondrial encephalopathies, particularly POLG-related disease. CoQ supplementation may be beneficial. Read More

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Sodium channel blockers for the treatment of epilepsy in CDKL5 deficiency disorder: Findings from a multicenter cohort.

Epilepsy Behav 2021 May 10;118:107946. Epub 2021 Apr 10.

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

Objective: This study was aimed to analyze the effectiveness of sodium channel blockers (SCBs) in CDKL5 deficiency disorder (CDD)-related epilepsy.

Methods: A retrospective, observational study was performed, including patients with CDD diagnosis evaluated between 2016 and 2019 at three tertiary Epilepsy Centers. Demographic, electroclinical and genetic features, as well as ASM treatments and their outcomes were analyzed, with special focus on SCBs. Read More

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[The efficacy and tolerability of extended release carbamazepine in adult patients with new-onset epilepsy using epileptiform activity index].

Zh Nevrol Psikhiatr Im S S Korsakova 2021 ;121(3):31-38

Sechenov First Moscow State Medical Univesity, Moscow, Russia.

Objective: To evaluate the efficacy and tolerability of extended release carbamazepine (finlepsin-retard and tegretol CR) in adult patients with new-onset focal epilepsy (FE) with the assessment of epileptiform activity index (EAI).

Material And Methods: The study included 62 patients (38 (61.3%) men and 24 (38. Read More

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Cation leak underlies neuronal excitability in an HCN1 developmental and epileptic encephalopathy.

Brain 2021 Apr 1. Epub 2021 Apr 1.

Florey Institute of Neuroscience and Mental Health, University of Melbourne, Parkville, VIC, Australia.

Pathogenic variants in HCN1 are associated with developmental and epileptic encephalopathies. The recurrent de novo HCN1 M305L pathogenic variant is associated with severe developmental impairment and drug-resistant epilepsy. We engineered the homologue Hcn1 M294L heterozygous knock-in (Hcn1M294L) mouse to explore the disease mechanism underlying an HCN1 developmental and epileptic encephalopathy. Read More

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Topography-Related EEG-fMRI in Surgically Confirmed Epileptic Foci: A Comparison to Spike-Related EEG-fMRI in Clinical Practice.

Brain Topogr 2021 May 17;34(3):373-383. Epub 2021 Mar 17.

Hotchkiss Brain Institute and Alberta Children's Hospital Research Institute, University of Calgary, Calgary, Canada.

EEG-fMRI has gained increasing importance in epilepsy pre-surgical diagnosis. However, 40-70% of EEG-fMRI recordings in patients lack interictal epileptiform discharges (IEDs) during the scan, which could be overcome by detecting matching topography maps. We tried to validate this method in clinical settings taking various electroclinical factors into consideration. Read More

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Sonographic hypoechogenicity of brainstem raphe nucleus is correlated with electroencephalographic spike frequency in patients with epilepsy.

Epilepsy Behav 2021 04 11;117:107884. Epub 2021 Mar 11.

Department of Neurology, Epilepsy and Headache Group, the First Affiliated Hospital of Anhui Medical University, Jixi Road 218, Hefei 230022, China. Electronic address:

Background: Brainstem raphe nucleus (BRN) hypoechogenicity in transcranial sonography (TCS) has been demonstrated in patients with major depression, possibly representing a sonographic manifestation of serotonergic dysfunction in depression. Most patients with epilepsy with comorbid depression exhibit hypoechogenic BRN in TCS. However, the role of BRN in the pathogenesis of epilepsy is unclear. Read More

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High-Density EEG in Current Clinical Practice and Opportunities for the Future.

J Clin Neurophysiol 2021 Mar;38(2):112-123

Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, U.S.A.

Summary: High-density EEG (HD-EEG) recordings use a higher spatial sampling of scalp electrodes than a standard 10-20 low-density EEG montage. Although several studies have demonstrated improved localization of the epileptogenic cortex using HD-EEG, widespread implementation is impeded by cost, setup and interpretation time, and lack of specific or sufficient procedural billing codes. Despite these barriers, HD-EEG has been in use at several institutions for years. Read More

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Ambulatory EEG Usefulness in Epilepsy Management.

J Clin Neurophysiol 2021 Mar;38(2):101-111

Department of Neurology, Mayo Clinic, Jacksonville, Florida, U.S.A.

Summary: Long-term video-EEG monitoring has been the gold standard for diagnosis of epileptic and nonepileptic events. Medication changes, safety, and a lack of recording EEG in one's habitual environment may interfere with diagnostic representation and subsequently affect management. Some spells defy standard EEG because of ultradian and circadian times of occurrence, manifest nocturnal expression of epileptiform activity, and require classification for clarifying diagnostic input to identify optimal treatment. 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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Recognition of interictal and ictal discharges on EEG. Focal vs generalized epilepsy.

Epilepsy Behav 2021 04 25;117:107830. Epub 2021 Feb 25.

Epilepsy, Detroit Medical Center, Wayne State University, United States.

Introduction: The differentiation between focal and generalized epilepsies based on clinical and electroencephalographic features is difficult and sometimes confusing.

Objective: To review the EEG findings in patients with focal epilepsy.

Methods: An extensive literature review was done. Read More

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Developmental and epileptic encephalopathies: recognition and approaches to care.

Epileptic Disord 2021 Feb;23(1):40-52

Paediatric Neurology Division, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, Cape Town, South Africa, Neurosciences Institute, University of Cape Town, South Africa.

The term "developmental and epileptic encephalopathy" (DEE) refers to when cognitive functions are influenced by both seizure and interictal epileptiform activity and the neurobiological process behind the epilepsy. Many DEEs are related to gene variants and the onset is typically during early childhood. In this setting, neurocognition, whilst not improved by seizure control, may benefit from some precision therapies. Read More

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February 2021

Temporal lobe dysfunction in late-onset epilepsy of unknown origin.

Epilepsy Behav 2021 04 18;117:107839. Epub 2021 Feb 18.

Department of Neurology, ASST S. Gerardo Hospital, University of Milano - Bicocca, Monza, Italy; Milan Center for Neuroscience (NeuroMI), Italy.

Objective: Epilepsy with onset in the adulthood is an increasing health problem, due to the progressive aging of the worldwide population. Whether the causes remain undetermined, the disease is defined as Late-Onset Epilepsy of Unknown origin (LOEU). The aim of this study was to evaluate the semiological, electroencephalographic, metabolic, and neuropsychological features of LOEU. Read More

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Bidirectional relationships of sleep and epilepsy in adults with epilepsy.

Epilepsy Behav 2021 03 6;116:107735. Epub 2021 Feb 6.

Sleep Disorders and Epilepsy Center, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA. Electronic address:

This targeted review addresses the best accepted and most intriguing recent observations on the complex relationships between sleep and epilepsy. Ten to 15% of all epilepsies are sleep-related. Included in these is sleep-related hypermotor epilepsy, renamed from nocturnal frontal lobe epilepsy by a 2016 consensus conference since 30% of cases are extra-frontal, seizures are related to sleep rather than clock time, and the predominant semiology is hypermotor. Read More

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Vortioxetine increases absence-like seizures in WAG/Rij rats but decreases penicillin- and pentylenetetrazole-induced seizures in Wistar rats.

Epilepsy Behav 2021 03 6;116:107797. Epub 2021 Feb 6.

Department of Physiology, Faculty of Medicine, University of Ondokuz Mayis Samsun, Turkey.

Aim: Depression is the major psychiatric disorder in patients with epilepsy. Vortioxetine is a novel antidepressant drug for the treatment of major depressive disorders. In the present study, effects of vortioxetine were evaluated in different experimental epilepsy models of rats. Read More

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Absolute spike frequency and different comorbidities in temporal lobe epilepsy.

Epilepsy Behav 2021 03 23;116:107730. Epub 2021 Jan 23.

Preventive Medicine Service, Army Health Unit, Santiago, Chile. Electronic address:

Objective: The objective of this study was to examine if the absolute number of interictal epileptiform discharges (IED) is related to the presence of different comorbidities and refractivity in patients with temporal lobe epilepsy.

Methods: Analysis with scalp EEG of the IED of 30 patients with temporal epilepsy. The analysis was performed in three selected periods of the record during N2-N3 sleep. Read More

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Seizures in autoimmune encephalitis-A systematic review and quantitative synthesis.

Epilepsia 2021 02 21;62(2):397-407. Epub 2021 Jan 21.

Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Objective: This study aimed to evaluate the proportion of patients with seizures and electroencephalography (EEG) abnormalities in autoimmune encephalitis (AE) and its most common subtypes.

Methods: This systematic review followed Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) standards and was registered with the International Prospective Register of Systematic Reviews (PROSPERO). We searched Medline All, Embase, and PsychINFO in Ovid from inception to June 2019 for articles pertaining to AE and seizure. Read More

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February 2021

Focal Sleep Spindle Deficits Reveal Focal Thalamocortical Dysfunction and Predict Cognitive Deficits in Sleep Activated Developmental Epilepsy.

J Neurosci 2021 Feb 19;41(8):1816-1829. Epub 2021 Jan 19.

Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts 02114

Childhood epilepsy with centrotemporal spikes (CECTS) is the most common focal epilepsy syndrome, yet the cause of this disease remains unknown. Now recognized as a mild epileptic encephalopathy, children exhibit sleep-activated focal epileptiform discharges and cognitive difficulties during the active phase of the disease. The association between the abnormal electrophysiology and sleep suggests disruption to thalamocortical circuits. Read More

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February 2021

Temporal patterns in the first unprovoked seizure.

Epilepsy Behav 2021 02 6;115:107625. Epub 2021 Jan 6.

Department of Medicine, St. Vincent's Hospital, University of Melbourne, Melbourne, Australia; Department of Neuroscience, Monash Medical Centre, Melbourne, Australia; School of Clinical Sciences at Monash Health, Department of Medicine, Monash University, Melbourne, Australia. Electronic address:

Objective: Cyclic phenomena in epilepsy are well recognized. We investigated a multicenter cohort of unprovoked first seizure presentations to determine whether seizures have a preponderance to occur in: a particular time of the day, a particular day of the week, a particular month of the year, day time versus night time, and wakefulness versus sleep.

Methods: We retrospectively studied adults who presented with a first-ever unprovoked seizure to the First Seizure Clinic at two tertiary centers in Australia. Read More

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February 2021

CDKL5 deficiency in forebrain glutamatergic neurons results in recurrent spontaneous seizures.

Epilepsia 2021 02 5;62(2):517-528. Epub 2021 Jan 5.

Institute of Neuroscience, State Key Laboratory of Neuroscience, Center for Excellence in Brain Science and Intelligence Technology, Chinese Academy of Sciences, Shanghai, China.

Objective: Mutations of the cyclin-dependent kinase-like 5 (CDKL5) gene cause severe neurodevelopmental disorders characterized by intractable epilepsy, intellectual disability, and autism. Multiple mouse models generated for mechanistic studies have exhibited phenotypes similar to some human pathological features, but none of the models has developed one of the major symptoms affecting CDKL5 deficiency disorder (CDD) patients: intractable recurrent seizures. As disrupted neuronal excitation/inhibition balance is closely associated with the activity of glutamatergic and γ-aminobutyric acidergic (GABAergic) neurons, our aim was to study the effect of the loss of CDKL5 in different types of neurons on epilepsy. Read More

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February 2021

Epilepsy and electroencephalogram evolution in YWHAG gene mutation: A new phenotype and review of the literature.

Am J Med Genet A 2021 03 4;185(3):901-908. Epub 2021 Jan 4.

Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

A male patient with a de novo mutation in the YWHAG gene and mild phenotype is presented. He had normal delivery and normal development, with normal speech and social milestones. At the age of 9 months, myoclonic seizures started, with generalized epileptiform discharges. Read More

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Spatiotemporal Patterns of High-Frequency Activity (80-170 Hz) in Long-Term Intracranial EEG.

Neurology 2021 02 22;96(7):e1070-e1081. Epub 2020 Dec 22.

From the Department of Biomedical Engineering (Z.C., D.B.G., A.N.B., P.J.K, M.J.C.), and Department of Medicine (Z.C., D.B.G., U.S., W.J.D., K.D., M.J.C., M.I.M.), St Vincent's Hospital, Department of Medicine (C.F.), Royal Melbourne Hospital, and Graeme Clark Institute (P.J.K., M.J.C.), The University of Melbourne, VIC, Australia; Cadence Neuroscience (K.L.), Redmond, WA; and 6 Seer Medical (M.I.M.), Melbourne, VIC, Australia.

Objective: To determine the utility of high-frequency activity (HFA) and epileptiform spikes as biomarkers for epilepsy, we examined the variability in their rates and locations using long-term ambulatory intracranial EEG (iEEG) recordings.

Methods: This study used continuous iEEG recordings obtained over an average of 1.4 years from 15 patients with drug-resistant focal epilepsy. Read More

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February 2021

Meta-analysis of EEG findings in patients with COVID-19.

Epilepsy Behav 2021 02 4;115:107682. Epub 2020 Dec 4.

Department of Pediatrics, Wayne State University, Detroit, USA; Department of Epileptology, Tohoku University Graduate School of Medicine, Sendai, Japan.

Objective: To perform a systematic review and meta-analysis to summarize and quantitatively evaluate the electroencephalogram (EEG) findings in patients with coronavirus disease 2019 (COVID-19).

Methods: The MEDLINE, CENTRAL, and ClinicalTrials.Gov databases were comprehensively assessed and searched for observational studies with EEG findings in patients with COVID-19. Read More

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February 2021

Forecasting seizure risk in adults with focal epilepsy: a development and validation study.

Lancet Neurol 2021 02 17;20(2):127-135. Epub 2020 Dec 17.

Sleep-Wake-Epilepsy Center, NeuroTec and Center for Experimental Neurology, Department of Neurology, Inselspital Bern, University Hospital, University of Bern, Bern, Switzerland; Wyss Center for Bio and Neuroengineering, Geneva, Switzerland. Electronic address:

Background: People with epilepsy are burdened with the apparent unpredictability of seizures. In the past decade, converging evidence from studies using chronic EEG (cEEG) revealed that epileptic brain activity shows robust cycles, operating over hours (circadian) and days (multidien). We hypothesised that these cycles can be leveraged to estimate future seizure probability, and we tested the feasibility of forecasting seizures days in advance. Read More

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February 2021

Lateralized Rhythmic Delta Activity in Patients With Infectious or Autoimmune Insular Lesions.

Clin EEG Neurosci 2021 Jan 14;52(1):61-65. Epub 2020 Oct 14.

Department of Neurology, Comprehensive Epilepsy Center, Johns Hopkins University, Baltimore, MD, USA.

Introduction: Lateralized rhythmic delta activity (LRDA) is a rare pattern on the ictal-interictal continuum (IIC) encountered in critically ill patients. Its association with acute seizures is yet to be fully explored. Insular involvement is a common finding in patients with infectious and autoimmune encephalitis. Read More

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January 2021

Factors correlated with intracranial interictal epileptiform discharges in refractory epilepsy.

Epilepsia 2021 02 17;62(2):481-491. Epub 2020 Dec 17.

Department of Neurology, Geisel School of Medicine at Dartmouth, Hanover, New Hampshire, USA.

Objective: This study was undertaken to evaluate the influence that subject-specific factors have on intracranial interictal epileptiform discharge (IED) rates in persons with refractory epilepsy.

Methods: One hundred fifty subjects with intracranial electrodes performed multiple sessions of a free recall memory task; this standardized task controlled for subject attention levels. We utilized a dominance analysis to rank the importance of subject-specific factors based on their relative influence on IED rates. Read More

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February 2021

Neurosteroids and Focal Epileptic Disorders.

Int J Mol Sci 2020 Dec 10;21(24). Epub 2020 Dec 10.

Montreal Neurological Institute-Hospital & Department of Neurology and Neurosurgery, 3801 University Street, Montreal, QC H3A 2B4, Canada.

Neurosteroids are a family of compounds that are synthesized in principal excitatory neurons and glial cells, and derive from the transformation of cholesterol into pregnenolone. The most studied neurosteroids-allopregnanolone and allotetrahydrodeoxycorticosterone (THDOC)-are known to modulate GABA receptor-mediated transmission, thus playing a role in controlling neuronal network excitability. Given the role of GABA signaling in epileptic disorders, neurosteroids have profound effects on seizure generation and play a role in the development of chronic epileptic conditions (i. Read More

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December 2020

Epilepsy and prion diseases: A narrative review.

Epilepsy Behav 2021 02 11;115:107630. Epub 2020 Dec 11.

Cognitive Function Clinic, The Walton Centre NHS Foundation Trust, Liverpool, United Kingdom.

Epileptic seizures have been described as one feature of prion diseases, but are an unusual clinical presentation. The aim of this narrative Review was to summarize current knowledge of epileptic seizures in the various forms of prion diseases, from a clinical perspective. Examination of the published literature identified no systematic studies; the evidence base is largely anecdotal, consisting mainly of case studies and small case series. Read More

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February 2021

Modulation of Brain Hyperexcitability: Potential New Therapeutic Approaches in Alzheimer's Disease.

Int J Mol Sci 2020 Dec 7;21(23). Epub 2020 Dec 7.

Cognitive Neurology Group, Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, University of Oxford, Oxford OX3 9DU, UK.

People with Alzheimer's disease (AD) have significantly higher rates of subclinical and overt epileptiform activity. In animal models, oligomeric Aβ amyloid is able to induce neuronal hyperexcitability even in the early phases of the disease. Such aberrant activity subsequently leads to downstream accumulation of toxic proteins, and ultimately to further neurodegeneration and neuronal silencing mediated by concomitant tau accumulation. Read More

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December 2020

Differences in course of illness between patients with bipolar II disorder with and without epileptiform discharges or other sharp activity on electroencephalograms: a cross-sectional study.

BMC Psychiatry 2020 12 7;20(1):582. Epub 2020 Dec 7.

Psychosomatic and CL Psychiatry, Division of Mental Health and Addiction, Oslo University Hospital, Oslo, Norway.

Background: A diagnosis of bipolar II disorder requires that the symptoms cannot be better explained by a medical condition. Epilepsy is in some cases associated with an affective syndrome mimicking an unstable bipolar II disorder. Epileptiform discharges on electroencephalograms (EEGs) are typical, but not pathognomonic, for epilepsy. Read More

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December 2020