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Epilepsia Apr 2017The International League Against Epilepsy (ILAE) presents a revised operational classification of seizure types. The purpose of such a revision is to recognize that some...
The International League Against Epilepsy (ILAE) presents a revised operational classification of seizure types. The purpose of such a revision is to recognize that some seizure types can have either a focal or generalized onset, to allow classification when the onset is unobserved, to include some missing seizure types, and to adopt more transparent names. Because current knowledge is insufficient to form a scientifically based classification, the 2017 Classification is operational (practical) and based on the 1981 Classification, extended in 2010. Changes include the following: (1) "partial" becomes "focal"; (2) awareness is used as a classifier of focal seizures; (3) the terms dyscognitive, simple partial, complex partial, psychic, and secondarily generalized are eliminated; (4) new focal seizure types include automatisms, behavior arrest, hyperkinetic, autonomic, cognitive, and emotional; (5) atonic, clonic, epileptic spasms, myoclonic, and tonic seizures can be of either focal or generalized onset; (6) focal to bilateral tonic-clonic seizure replaces secondarily generalized seizure; (7) new generalized seizure types are absence with eyelid myoclonia, myoclonic absence, myoclonic-atonic, myoclonic-tonic-clonic; and (8) seizures of unknown onset may have features that can still be classified. The new classification does not represent a fundamental change, but allows greater flexibility and transparency in naming seizure types.
Topics: Epilepsy; Humans; International Agencies; Seizures; Societies, Medical; Terminology as Topic
PubMed: 28276060
DOI: 10.1111/epi.13670 -
American Family Physician Apr 2019A febrile seizure is a seizure occurring in a child six months to five years of age that is accompanied by a fever (100.4°F or greater) without central nervous system... (Review)
Review
A febrile seizure is a seizure occurring in a child six months to five years of age that is accompanied by a fever (100.4°F or greater) without central nervous system infection. Febrile seizures are classified as simple or complex. A complex seizure lasts 15 minutes or more, is associated with focal neurologic findings, or recurs within 24 hours. The cause of febrile seizures is likely multifactorial. Viral illnesses, certain vaccinations, and genetic predisposition are common risk factors that may affect a vulnerable, developing nervous system under the stress of a fever. Children who have a simple febrile seizure and are well-appearing do not require routine diagnostic testing (laboratory tests, neuroimaging, or electroencephalography), except as indicated to discern the cause of the fever. For children with complex seizures, the neurologic examination should guide further evaluation. For seizures lasting more than five minutes, a benzodiazepine should be administered. Febrile seizures are not associated with increased long-term mortality or negative effects on future academic progress, intellect, or behavior. Children with febrile seizures are more likely to have recurrent febrile seizures. However, given the benign nature of febrile seizures, the routine use of antiepileptics is not indicated because of adverse effects of these medications. The use of antipyretics does not decrease the risk of febrile seizures, although rectal acetaminophen reduced the risk of short-term recurrence following a febrile seizure. Parents should be educated on the excellent prognosis of children with febrile seizures and provided with practical guidance on home management of seizures.
Topics: Antipyretics; Humans; Neuroimaging; Prognosis; Recurrence; Risk Factors; Seizures, Febrile
PubMed: 30932454
DOI: No ID Found -
The Cornell Veterinarian Apr 1981Recent developments in the understanding of the cellular events in focal seizures are described. This is followed by a discussion of the pathogenesis of neuronal... (Review)
Review
Recent developments in the understanding of the cellular events in focal seizures are described. This is followed by a discussion of the pathogenesis of neuronal hyperexcitability, the mechanism of spread of the seizure discharge and the mechanisms involved in seizure termination. The pathophysiology of generalized seizures is described in terms of theories of the site of origin of the seizure discharge and possible mechanisms of the clinical phenomena associated with this discharge.
Topics: Animals; Brain; Cats; Cell Membrane; Cerebral Cortex; Electric Stimulation; Electroencephalography; Epilepsies, Partial; Epilepsy; Humans; Neural Pathways; Neurons; Seizures; Synaptic Transmission
PubMed: 6260428
DOI: No ID Found -
JAMA Neurology Apr 2021Focal epilepsy is characterized by the cyclical recurrence of seizures, but, to our knowledge, the prevalence and patterns of seizure cycles are unknown.
IMPORTANCE
Focal epilepsy is characterized by the cyclical recurrence of seizures, but, to our knowledge, the prevalence and patterns of seizure cycles are unknown.
OBJECTIVE
To establish the prevalence, strength, and temporal patterns of seizure cycles over timescales of hours to years.
DESIGN, SETTING, AND PARTICIPANTS
This retrospective cohort study analyzed data from continuous intracranial electroencephalography (cEEG) and seizure diaries collected between January 19, 2004, and May 18, 2018, with durations up to 10 years. A total of 222 adults with medically refractory focal epilepsy were selected from 256 total participants in a clinical trial of an implanted responsive neurostimulation device. Selection was based on availability of cEEG and/or self-reports of disabling seizures.
EXPOSURES
Antiseizure medications and responsive neurostimulation, based on clinical indications.
MAIN OUTCOMES AND MEASURES
Measures involved (1) self-reported daily seizure counts, (2) cEEG-based hourly counts of electrographic seizures, and (3) detections of interictal epileptiform activity (IEA), which fluctuates in daily (circadian) and multiday (multidien) cycles. Outcomes involved descriptive characteristics of cycles of IEA and seizures: (1) prevalence, defined as the percentage of patients with a given type of seizure cycle; (2) strength, defined as the degree of consistency with which seizures occur at certain phases of an underlying cycle, measured as the phase-locking value (PLV); and (3) seizure chronotypes, defined as patterns in seizure timing evident at the group level.
RESULTS
Of the 222 participants, 112 (50%) were male, and the median age was 35 years (range, 18-66 years). The prevalence of circannual (approximately 1 year) seizure cycles was 12% (24 of 194), the prevalence of multidien (approximately weekly to approximately monthly) seizure cycles was 60% (112 of 186), and the prevalence of circadian (approximately 24 hours) seizure cycles was 89% (76 of 85). Strengths of circadian (mean [SD] PLV, 0.34 [0.18]) and multidien (mean [SD] PLV, 0.34 [0.17]) seizure cycles were comparable, whereas circannual seizure cycles were weaker (mean [SD] PLV, 0.17 [0.10]). Across individuals, circadian seizure cycles showed 5 peaks: morning, mid-afternoon, evening, early night, and late night. Multidien cycles of IEA showed peak periodicities centered around 7, 15, 20, and 30 days. Independent of multidien period length, self-reported and electrographic seizures consistently occurred during the days-long rising phase of multidien cycles of IEA.
CONCLUSIONS AND RELEVANCE
Findings in this large cohort establish the high prevalence of plural seizure cycles and help explain the natural variability in seizure timing. The results have the potential to inform the scheduling of diagnostic studies, the delivery of time-varying therapies, and the design of clinical trials in epilepsy.
Topics: Adolescent; Adult; Aged; Circadian Rhythm; Cohort Studies; Electrocorticography; Epilepsies, Partial; Female; Humans; Implantable Neurostimulators; Male; Middle Aged; Retrospective Studies; Seizures; Young Adult
PubMed: 33555292
DOI: 10.1001/jamaneurol.2020.5370 -
Brain and Behavior Sep 2022Epilepsy is one of the most common neurological conditions worldwide. As a chronic condition, epilepsy imposes a significant burden on people with epilepsy and society.... (Review)
Review
BACKGROUND
Epilepsy is one of the most common neurological conditions worldwide. As a chronic condition, epilepsy imposes a significant burden on people with epilepsy and society. We aimed to assess the burden and unmet need of individuals with epilepsy and their caregivers, focusing on focal seizures, the main type of seizure in adults and children.
METHODS
A targeted evidence review of the burden of epilepsy, focusing on focal seizures, was conducted to identify articles reporting: epidemiology, mortality, morbidity, quality of life (QoL), and costs.
RESULTS
Focal seizures affect up to ∼61% of people with epilepsy. They are associated with an increased risk of injury and premature death than the general population. People with epilepsy also have high comorbidity, particularly depression, anxiety, and cognitive impairments. Higher seizure frequency, adverse treatment events, and employment concerns reduce QoL. A reduction in caregivers' QoL is also often reported. Epilepsy requires long-term treatment accounting for high individual costs. Hospitalizations and antiseizure medications (ASMs) are the leading cost drivers of inpatient management and indirect costs with high unemployment rates, particularly in drug-resistant populations. Despite the advent of new treatments, a high unmet need remains unaddressed; approximately 40% of people with epilepsy are drug-resistant, further increasing the risks associated with epilepsy.
CONCLUSIONS
Our findings highlight a substantial burden of illness and unmet needs in individuals with focal seizures, especially those with drug-resistant epilepsy. Suboptimal treatment options negatively impact QoL and, consequently, a sizeable economic burden indicating the need for new treatments and prioritizing this condition.
Topics: Adult; Anticonvulsants; Child; Drug Resistant Epilepsy; Epilepsy; Humans; Quality of Life; Seizures
PubMed: 36017757
DOI: 10.1002/brb3.2589 -
BMJ Clinical Evidence Jan 2014Simple febrile seizures are generalised in onset and have a brief duration. The American Academy of Pediatrics defines this brief duration to be <15 minutes; whereas, in... (Review)
Review
INTRODUCTION
Simple febrile seizures are generalised in onset and have a brief duration. The American Academy of Pediatrics defines this brief duration to be <15 minutes; whereas, in the UK, a maximum duration of 10 minutes is used. Simple febrile seizures do not occur more than once in 24 hours and resolve spontaneously. Complex febrile seizures are longer lasting, have focal symptoms (at onset or during the seizure), and can recur within 24 hours or within the same febrile illness. This review only deals with simple febrile seizures. About 2% to 5% of children in the US and Western Europe, and 6% to 9% of infants and children in Japan, will have experienced at least one febrile seizure by the age of 5 years. A very small number of children with simple febrile seizures may develop afebrile seizures, but simple febrile seizures are not associated with any permanent neurological deficits.
METHODS AND OUTCOMES
We conducted a systematic review and aimed to answer the following clinical question: What are the effects of treatments given during episodes of fever in children (aged 6 months to 5 years) with one or more previous simple febrile seizures? We searched: Medline, Embase, The Cochrane Library, and other important databases up to July 2013 (Clinical Evidence reviews are updated periodically, please check our website for the most up-to-date version of this review). We included harms alerts from relevant organisations such as the US Food and Drug Administration (FDA) and the UK Medicines and Healthcare products Regulatory Agency (MHRA).
RESULTS
We found 4 RCTs or systematic reviews of RCTs that met our inclusion criteria. We performed a GRADE evaluation of the quality of evidence for interventions.
CONCLUSIONS
In this systematic review, we present information relating to the effectiveness and safety of the following interventions: intermittent anticonvulsants (clobazam, diazepam, lorazepam), antipyretic drug treatments (paracetamol, ibuprofen), and conservative measures (watchful waiting, physical antipyretic measures [tepid sponging, removing clothes, cooling room, direct fanning of child]).
Topics: Anticonvulsants; Europe; Fever; Humans; Seizures, Febrile
PubMed: 24484859
DOI: No ID Found -
Epilepsia Apr 2010The International League Against Epilepsy (ILAE) Commission on Classification and Terminology has revised concepts, terminology, and approaches for classifying seizures...
The International League Against Epilepsy (ILAE) Commission on Classification and Terminology has revised concepts, terminology, and approaches for classifying seizures and forms of epilepsy. Generalized and focal are redefined for seizures as occurring in and rapidly engaging bilaterally distributed networks (generalized) and within networks limited to one hemisphere and either discretely localized or more widely distributed (focal). Classification of generalized seizures is simplified. No natural classification for focal seizures exists; focal seizures should be described according to their manifestations (e.g., dyscognitive, focal motor). The concepts of generalized and focal do not apply to electroclinical syndromes. Genetic, structural-metabolic, and unknown represent modified concepts to replace idiopathic, symptomatic, and cryptogenic. Not all epilepsies are recognized as electroclinical syndromes. Organization of forms of epilepsy is first by specificity: electroclinical syndromes, nonsyndromic epilepsies with structural-metabolic causes, and epilepsies of unknown cause. Further organization within these divisions can be accomplished in a flexible manner depending on purpose. Natural classes (e.g., specific underlying cause, age at onset, associated seizure type), or pragmatic groupings (e.g., epileptic encephalopathies, self-limited electroclinical syndromes) may serve as the basis for organizing knowledge about recognized forms of epilepsy and facilitate identification of new forms.
Topics: Epilepsy; Humans; International Agencies; Seizures; Syndrome; Terminology as Topic
PubMed: 20196795
DOI: 10.1111/j.1528-1167.2010.02522.x -
The Lancet. Neurology Jul 2017People with epilepsy who became seizure-free while taking antiepileptic drugs might consider discontinuing their medication, with the possibility of increased quality of... (Meta-Analysis)
Meta-Analysis Review
Individualised prediction model of seizure recurrence and long-term outcomes after withdrawal of antiepileptic drugs in seizure-free patients: a systematic review and individual participant data meta-analysis.
BACKGROUND
People with epilepsy who became seizure-free while taking antiepileptic drugs might consider discontinuing their medication, with the possibility of increased quality of life because of the elimination of adverse events. The risk with this action, however, is seizure recurrence. The objectives of our study were to identify predictors of seizure recurrence and long-term seizure outcomes and to produce nomograms for estimation of individualised outcomes.
METHODS
We did a systematic review and meta-analysis, and identified eligible articles and candidate predictors, using PubMed and Embase databases with a last update on Nov 6, 2014. Eligible articles had to report on cohorts of patients with epilepsy who were seizure-free and had started withdrawal of antiepileptic drugs; articles also had to contain information regarding seizure recurrences during and after withdrawal. We excluded surgical cohorts, reports with fewer than 30 patients, and reports on acute symptomatic seizures because these topics were beyond the scope of our objective. Risk of bias was assessed using the Quality in Prognosis Studies system. Data analysis was based on individual participant data. Survival curves and proportional hazards were computed. The strongest predictors were selected with backward selection. Models were converted to nomograms and a web-based tool to determine individual risks.
FINDINGS
We identified 45 studies with 7082 patients; ten studies (22%) with 1769 patients (25%) were included in the meta-analysis. Median follow-up was 5·3 years (IQR 3·0-10·0, maximum 23 years). Prospective and retrospective studies and randomised controlled trials were included, covering non-selected and selected populations of both children and adults. Relapse occurred in 812 (46%) of 1769 patients; 136 (9%) of 1455 for whom data were available had seizures in their last year of follow-up, suggesting enduring seizure control was not regained by this timepoint. Independent predictors of seizure recurrence were epilepsy duration before remission, seizure-free interval before antiepileptic drug withdrawal, age at onset of epilepsy, history of febrile seizures, number of seizures before remission, absence of a self-limiting epilepsy syndrome, developmental delay, and epileptiform abnormality on electroencephalogram (EEG) before withdrawal. Independent predictors of seizures in the last year of follow-up were epilepsy duration before remission, seizure-free interval before antiepileptic drug withdrawal, number of antiepileptic drugs before withdrawal, female sex, family history of epilepsy, number of seizures before remission, focal seizures, and epileptiform abnormality on EEG before withdrawal. Adjusted concordance statistics were 0·65 (95% CI 0·65-0·66) for predicting seizure recurrence and 0·71 (0·70-0·71) for predicting long-term seizure freedom. Validation was stable across the individual study populations.
INTERPRETATION
We present evidence-based nomograms with robust performance across populations of children and adults. The nomograms facilitate prediction of outcomes following drug withdrawal for the individual patient, including both the risk of relapse and the chance of long-term freedom from seizures. The main limitations were the absence of a control group continuing antiepileptic drug treatment and a consistent definition of long-term seizure freedom.
FUNDING
Epilepsiefonds.
Topics: Adult; Anticonvulsants; Child; Humans; Outcome Assessment, Health Care; Recurrence; Remission Induction; Seizures
PubMed: 28483337
DOI: 10.1016/S1474-4422(17)30114-X -
JCI Insight Dec 2022Developmental and epileptic encephalopathies (DEEs) are characterized by pharmaco-resistant seizures with concomitant intellectual disability. Epilepsy of infancy with...
Developmental and epileptic encephalopathies (DEEs) are characterized by pharmaco-resistant seizures with concomitant intellectual disability. Epilepsy of infancy with migrating focal seizures (EIMFS) is one of the most severe of these syndromes. De novo variants in ion channels, including gain-of-function variants in KCNT1, which encodes for sodium activated potassium channel protein KNa1.1, have been found to play a major role in the etiology of EIMFS. Here, we test a potential precision therapeutic approach in KCNT1-associated DEE using a gene-silencing antisense oligonucleotide (ASO) approach. We generated a mouse model carrying the KCNT1 p.P924L pathogenic variant; only the homozygous animals presented with the frequent, debilitating seizures and developmental compromise that are seen in patients. After a single intracerebroventricular bolus injection of a Kcnt1 gapmer ASO in symptomatic mice at postnatal day 40, seizure frequency was significantly reduced, behavioral abnormalities improved, and overall survival was extended compared with mice treated with a control ASO (nonhybridizing sequence). ASO administration at neonatal age was also well tolerated and effective in controlling seizures and extending the life span of treated animals. The data presented here provide proof of concept for ASO-based gene silencing as a promising therapeutic approach in KCNT1-associated epilepsies.
Topics: Mice; Animals; Brain Diseases; Seizures
PubMed: 36173683
DOI: 10.1172/jci.insight.146090 -
Epilepsia Sep 1998We propose an epileptic seizure classification based exclusively on ictal semiology. In this semiological seizure classification (SSC), seizures are classified as...
We propose an epileptic seizure classification based exclusively on ictal semiology. In this semiological seizure classification (SSC), seizures are classified as follows: a. Auras are ictal manifestations having sensory, psychosensory, and experiential symptoms. b. Autonomic seizures are seizures in which the main ictal manifestations are objectively documented autonomic alterations. c. "Dialeptic" seizures have as their main ictal manifestations an alteration of consciousness that is independent of ictal EEG manifestations. The new term "dialeptic" seizure has been coined to differentiate this concept from absence seizures (dialeptic seizures with a generalized ictal EEG) and complex partial seizures (dialeptic seizures with a focal ictal EEG). d. Motor seizures are characterized mainly by motor symptoms and are subclassified as simple or complex. Simple motor seizures are characterized by simple, unnatural movements that can be elicited by electrical stimulation of the primary and supplementary motor area (myoclonic, tonic, clonic and tonic-clonic, versive). Complex motor seizures are characterized by complex motor movements that resemble natural movements but that occur in an inappropriate setting ("automatisms"). e. Special seizures include seizures characterized by "negative" features (atonic, astatic, hypomotor, akinetic, and aphasic seizures). The SSC identifies in detail the somatotopic distribution of the ictal semiology as well as the seizure evolution. The advantages of a pure SSC, as opposed to the current classification of the International League Against Epilepsy (ILAE), which is actually a classification of electroclinical syndromes, are discussed.
Topics: Electroencephalography; Epilepsy; Humans; Seizures; Syndrome; Terminology as Topic
PubMed: 9738682
DOI: 10.1111/j.1528-1157.1998.tb01452.x