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Journal of Clinical Neurophysiology :... Mar 2024Among the many fears associated with seizures, patients with epilepsy are greatly frustrated and distressed over seizure's apparent unpredictable occurrence. However,... (Review)
Review
Among the many fears associated with seizures, patients with epilepsy are greatly frustrated and distressed over seizure's apparent unpredictable occurrence. However, increasing evidence have emerged over the years to support that seizure occurrence is not a random phenomenon as previously presumed; it has a cyclic rhythm that oscillates over multiple timescales. The pattern in rises and falls of seizure rate that varies over 24 hours, weeks, months, and years has become a target for the development of innovative devices that intend to detect, predict, and forecast seizures. This article will review the different tools and devices available or that have been previously studied for seizure detection, prediction, and forecasting, as well as the associated challenges and limitations with the utilization of these devices. Although there is strong evidence for rhythmicity in seizure occurrence, very little is known about the mechanism behind this oscillation. This article concludes with early insights into the regulations that may potentially drive this cyclical variability and future directions.
Topics: Humans; Seizures; Emotions
PubMed: 38436388
DOI: 10.1097/WNP.0000000000001045 -
Epilepsy & Behavior : E&B Feb 2024Inherited metabolic epilepsies (IMEs) represent inherited metabolic disorders predominately presenting with seizures. While most IMEs are currently managed with... (Review)
Review
Inherited metabolic epilepsies (IMEs) represent inherited metabolic disorders predominately presenting with seizures. While most IMEs are currently managed with symptomatic and supportive therapies, some are amenable to disorder-specific targeted treatments. In most cases, these treatments are effective only if given in a narrow time window early in the lives of affected patients. Hence, prompt recognition of treatable inherited metabolic epilepsies at an early age and as soon as symptoms appear has paramount importance. Herein, we provide an overview of inherited metabolic epilepsies, which presently have established targeted treatments showing clinical efficacy in reducing seizure burden and improving neurodevelopmental outcomes. These therapeutic modalities range from specific diets, vitamins, and supplementation of organic compounds to synthetic pharmacological agents and novel genetic-based therapies that alter the biochemical pathways of these disorders at the cellular or molecular level, steering them to their normal function.
Topics: Humans; Epilepsy; Metabolic Diseases; Seizures; Treatment Outcome; Vitamins
PubMed: 38237465
DOI: 10.1016/j.yebeh.2024.109621 -
Handbook of Clinical Neurology 2024Seizures are a common feature of autoimmune encephalitis and are especially prevalent in patients with the commonest autoantibodies, against LGI1, CASPR2 and the NMDA,... (Review)
Review
Seizures are a common feature of autoimmune encephalitis and are especially prevalent in patients with the commonest autoantibodies, against LGI1, CASPR2 and the NMDA, GABA, and GABA receptors. In this chapter, we discuss the classification, clinical, investigation, and treatment aspects of patients with these, and other autoantibody-mediated and -associated, illnesses. We highlight distinctive and common seizure semiologies which, often alongside other features we outline, can help the clinical diagnosis of an autoantibody-associated syndrome. Next, we classify these syndromes by either focusing on whether they represent underlying causative autoantibodies or T-cell-mediated syndromes and on the distinction between acute symptomatic seizures and a more enduring tendency to autoimmune-associated epilepsy, a practical and valuable distinction for both patients and clinicians which relates to the pathogenesis. We emphasize the more effective immunotherapy response in patients with causative autoantibodies, and discuss the emerging evidence for various first-, second-, and third-line immunotherapies. Finally, we highlight available clinical rating scales which can guide autoantibody testing and immunotherapy in patients with seizures of unknown etiology. Throughout, we relate the clinical and therapeutic observations to the immunobiology and neuroscience which drive these seizures.
Topics: Humans; Seizures; Epilepsy; Encephalitis; Autoantibodies; gamma-Aminobutyric Acid
PubMed: 38494275
DOI: 10.1016/B978-0-12-823912-4.00009-8 -
Epilepsy Research Dec 2023Secondary epileptogenesis is a theory that hypothesizes that uncontrolled seizures in people with epilepsy lead to the development of new sites of seizure onset. This... (Review)
Review
Secondary epileptogenesis is a theory that hypothesizes that uncontrolled seizures in people with epilepsy lead to the development of new sites of seizure onset. This process has often been cited when people experience a new seizure type after a period of poor seizure control. The theory proposes that repeated seizures induce changes in regions of the brain that are regularly recruited into the seizure. These hypothetical changes can then lead to a new, independent seizure onset zone. The concept is based on a number of clinical observations which secondary epileptogenesis could explain. However there are alternative explanations from the clinic as well as from the laboratory that call the process into question. In this review some of the observations that have been used to support the theory will be reviewed, and the many counterarguments will be presented. At this time there is little evidence to support secondary epileptogenesis and much to refute it.
Topics: Humans; Kindling, Neurologic; Seizures; Epilepsy; Brain
PubMed: 37336709
DOI: 10.1016/j.eplepsyres.2023.107179 -
Epilepsia Aug 2023This study was undertaken to evaluate perampanel (PER) when used under real-world conditions to treat people with idiopathic generalized epilepsy (IGE) included in the... (Clinical Trial)
Clinical Trial
OBJECTIVE
This study was undertaken to evaluate perampanel (PER) when used under real-world conditions to treat people with idiopathic generalized epilepsy (IGE) included in the PERaMpanel pooled analysIs of effecTiveness and tolerability (PERMIT) study.
METHODS
The multinational, retrospective, pooled analysis PERMIT explored the use of PER in people with focal and generalized epilepsy treated in clinical practice across 17 countries. This subgroup analysis included PERMIT participants with IGE. Time points for retention and effectiveness measurements were 3, 6, and 12 months (last observation carried forward, defined as "last visit," was also applied to effectiveness). Effectiveness was evaluated by seizure type (total seizures, generalized tonic-clonic seizures [GTCS], myoclonic seizures, absence seizures) and included ≥50% responder rate and seizure freedom rate (defined as no seizures since at least the previous visit). Safety/tolerability was monitored throughout PER treatment and evaluated by documenting the incidence of adverse events (AEs), including psychiatric AEs and those leading to treatment discontinuation.
RESULTS
The Full Analysis Set included 544 people with IGE (51.9% women, mean age = 33.3 years, mean epilepsy duration = 18.1 years). At 3, 6, and 12 months, 92.4%, 85.5%, and 77.3% of participants were retained on PER treatment, respectively (Retention Population, n = 497). At the last visit, responder and seizure freedom rates were, respectively, 74.2% and 54.6% (total seizures), 81.2% and 61.5% (GTCS), 85.7% and 66.0% (myoclonic seizures), and 90.5% and 81.0% (absence seizures) (Effectiveness Population, n = 467). AEs occurred in 42.9% of patients and included irritability (9.6%), dizziness/vertigo (9.2%), and somnolence (6.3%) (Tolerability Population, n = 520). Treatment discontinuation due to AEs was 12.4% over 12 months.
SIGNIFICANCE
This subgroup analysis of the PERMIT study demonstrated the effectiveness and good tolerability of PER in people with IGE when administered under everyday clinical practice conditions. These findings are in line with clinical trial evidence, supporting PER's use as broad-spectrum antiseizure medication for the treatment of IGE.
Topics: Adult; Female; Humans; Male; Anticonvulsants; Drug Therapy, Combination; Epilepsies, Myoclonic; Epilepsy, Absence; Epilepsy, Generalized; Immunoglobulin E; Pyridones; Retrospective Studies; Seizures; Treatment Outcome
PubMed: 37114853
DOI: 10.1111/epi.17631 -
Pediatric Annals Oct 2023
Topics: Humans; Child; Seizures
PubMed: 37820702
DOI: 10.3928/19382359-20230829-07 -
Epilepsy Research Jul 2023Epilepsy with eyelid myoclonia (EEM) is a generalized epilepsy syndrome with childhood-onset and 2:1 female predominance that consists of: 1. eyelid myoclonia with or... (Review)
Review
Epilepsy with eyelid myoclonia (EEM) is a generalized epilepsy syndrome with childhood-onset and 2:1 female predominance that consists of: 1. eyelid myoclonia with or without absence seizures, 2. eye closure induced seizures or EEG paroxysms, 3. clinical or EEG photosensitivity. While eyelid myoclonia is the disease hallmark, other seizure types, including absence seizures and generalized tonic-clonic seizures, may be present. It is thought to have a genetic etiology, and around one-third of patients may have a positive family history of epilepsy. Recently, specific genetic mutations have been recognized in a minority patients, including in SYNGAP1, NEXMIF, RORB, and CHD2 genes. There are no randomized controlled trials in EEM, and the management literature is largely restricted to small retrospective studies. Broad-spectrum antiseizure medications such as valproate, levetiracetam, lamotrigine, and benzodiazepines are typically used. Seizures typically persist into adulthood, and drug-resistant epilepsy is reported in over 50%.
Topics: Humans; Female; Child; Male; Retrospective Studies; Anticonvulsants; Epilepsy, Generalized; Seizures; Myoclonus; Epilepsy, Absence; Eyelids; Electroencephalography
PubMed: 37121024
DOI: 10.1016/j.eplepsyres.2023.107147 -
Epileptic Disorders : International... Aug 2023To characterize the duration of seizures and inter-seizure intervals in focal status epilepticus (SE). (Review)
Review
OBJECTIVE
To characterize the duration of seizures and inter-seizure intervals in focal status epilepticus (SE).
METHODS
We reviewed consecutive scalp EEG recordings from adult patients who were admitted for a first episode of focal status epilepticus. We identified electrographic seizure duration and inter-seizure intervals in the first diagnostic pretreatment EEG. We also reviewed isolated focal self-limiting seizures in epilepsy patients, as a comparison group for seizure duration.
RESULTS
We recorded 307 focal seizures in 100 consecutive focal SE episodes, with a median seizure duration of 107 s (IQR: 54-186), and 134 isolated focal self-limiting seizures in 42 epilepsy patients, with a median duration of 59 s (IQR: 30-90; p < .001). The only clinical feature of SE that significantly increased seizure duration was acute symptomatic etiology. In SE, 15% and 7% of seizures lasted longer than 300 and 600 s, respectively (t1 of the actual definition for tonic-clonic and focal SE), while only 1% of self-limiting seizures lasted longer than 300 s, and none lasted longer than 600 s. The analysis of inter-seizure intervals in SE with multiple seizures showed that 50% of the inter-seizure periods were shorter than 60 s, and 95% were shorter than 540 s (9 min). Patients who had an increase in seizure duration (last versus first) of at least 1.4 times showed an increased 30-day mortality.
SIGNIFICANCE
Focal seizures within a SE episode showed a wide range of duration, partly overlapping with the duration of focal self-limiting seizures but with a longer median duration. Inter-seizure intervals within an episode of SE were shorter than 1 min in 50% of the seizures and never lasted more than 10 min. Finally, an increase in seizure duration could represent an "electrophysiological biomarker" of a more severe SE episode, which may require more aggressive and rapid treatment.
Topics: Adult; Humans; Status Epilepticus; Seizures; Epilepsy; Epilepsia Partialis Continua; Electroencephalography
PubMed: 37170072
DOI: 10.1002/epd2.20071 -
Journal of Clinical Neurophysiology :... Jan 2024Presurgical evaluations to plan intracranial EEG implantations or surgical therapies at most epilepsy centers in the United States currently depend on the visual... (Review)
Review
Presurgical evaluations to plan intracranial EEG implantations or surgical therapies at most epilepsy centers in the United States currently depend on the visual inspection of EEG traces. Such analysis is inadequate and does not exploit all the localizing information contained in scalp EEG. Various types of EEG source modeling or imaging can provide sublobar localization of spike and seizure sources in the brain, and the software to do this with typical long-term monitoring EEG data are available to all epilepsy centers. This article reviews the fundamentals of EEG voltage fields that are used in EEG source imaging, the strengths and weakness of dipole and current density source models, the clinical situations where EEG source imaging is most useful, and the particular strengths of EEG source imaging for various cortical areas where spike/seizure sources are likely.
Topics: Humans; Brain; Electrocorticography; Electroencephalography; Seizures; Epilepsy
PubMed: 38181386
DOI: 10.1097/WNP.0000000000001018 -
Pediatric Neurology Nov 2023Typical absence seizures (TAS) are seen in idiopathic generalized epilepsy. Electroencephalography (EEG) contributes to syndrome characterization and counseling in an...
BACKGROUND
Typical absence seizures (TAS) are seen in idiopathic generalized epilepsy. Electroencephalography (EEG) contributes to syndrome characterization and counseling in an area where genetics does not currently play a significant role. Prominent interictal EEG findings are seen in juvenile absence epilepsy (JAE) and are thus thought to be associated with less favorable outcome in any TAS case despite lack of evidence. Our study evaluates EEG findings and their association with seizure outcomes in children with TAS.
METHODS
Retrospective cohort study of 123 children over 10 years with extensive EEG analysis and medical record review. Phone interviews ascertained longer-term outcomes. EEG reviewers were unaware of outcomes.
RESULTS
Total cohort included 123 children with phone review completed in 98. Median follow-up was 5 years 9 months. Seizure freedom was seen in 59% off antiseizure medicines (ASMs). Interictal findings included focal discharges in 29%, fragments of spike-wave (SW) discharges in 82.1%, and generalized interictal discharges in 63.4%. Interictal SW was more likely in those who slept (100%, 18 of 18) versus those who did not (57%, 60 of 105) (P < 0.001). Outcome analysis found no associations between focal or generalized interictal findings and seizure freedom, relapse off ASM, occurrence of other seizure types, or response to first ASM.
CONCLUSION
Focal and generalized interictal EEG discharges are common in children with TAS and are not associated with poorer outcomes. These interictal findings were traditionally associated with JAE rather than childhood absence epilepsy and were thus believed to be associated with potentially poorer outcome, which is probably not the case.
Topics: Child; Humans; Retrospective Studies; Seizures; Electroencephalography; Epilepsy, Generalized; Epilepsy, Absence
PubMed: 37666206
DOI: 10.1016/j.pediatrneurol.2023.08.004