Seizure emergencies experienced by patients with epilepsy include status epilepticus and seizure clusters. Although an accepted definition of status epilepticus exists, no clear consensus definition of seizure clusters has emerged; this is further complicated by the appearance in the literature of various empirically based definitions that have been developed for clinical trial study designs. In general, patients with intractable epilepsy have been shown to have a significant risk for acute episodes of increased seizure activity called seizure clusters (also referred to as acute repetitive seizures, among other terms) that differ from their usual seizure pattern. Duration (e.g., number of hours or days) is often included in the definition of a seizure cluster; however, the duration may vary among patients, with some seizure clusters lasting ≥24 h and requiring long-acting treatment for this period. In addition to seizure cluster duration, the time between seizures and possible acceleration in seizure frequency during the cluster may be important variables. The recognition and treatment of seizure clusters require urgent action because episodes that are not quickly and appropriately treated may lead to injury or progress to status epilepticus or potentially death. Most seizure clusters occur outside a medical facility (in the community) and treatment is usually administered by nonmedical individuals; therefore, health care providers may benefit from a clear description of these potential seizure emergencies that they can then use to educate patients and caregivers on the prompt and appropriate identification of seizure clusters and administration of rescue therapy. Here we explore why greater uniformity is needed in the discussion of seizure clusters. This exploration examines epidemiologic studies of seizure clusters and status epilepticus, inconsistencies in nomenclature and definitions for seizure clusters, practical application of seizure cluster terminology, and the potential use of acute seizure action plans and patient-specific individualized definitions in the clinical setting.
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http://dx.doi.org/10.1111/epi.17346 | DOI Listing |
Neurobiol Dis
January 2025
Department of Neurology, the First Affiliated Hospital of Guangxi Medical University, Guangxi Medical University, Nanning, Guangxi, China. Electronic address:
One of the underlying mechanisms of epilepsy (EP), a brain disease characterized by recurrent seizures, is considered to be cell death. Disulfidptosis, a proposed novel cell death mechanism, is thought to play a part in the pathogenesis of epilepsy, but the exact role is unclear. The gene expression omnibus series (GSE) 33,000 and GSE63808 datasets were used to search for differentially expressed disulfidptosis-related molecules (DE-DRMs).
View Article and Find Full Text PDFEpilepsia
January 2025
Division of Pediatric Neurology and Developmental Medicine, Department of Pediatrics, Duke University, Durham, North Carolina, USA.
Objective: This study was undertaken to test the following hypotheses in the Atp1a3 mouse (which carries the most common human ATP1A3 (the major subunit of the neuronal Na/K-adenosine triphosphatase [ATPase]) mutation, D801N): sudden unexpected death in epilepsy (SUDEP) occurs during seizures and is due to terminal apneas in some and due to lethal cardiac arrhythmias in others; and Atp1a3 mice have central cardiorespiratory dysregulation and abnormal respiratory drive.
Methods: Comparison was made of littermate wild-type and Atp1a3 groups using (1) simultaneous in vivo video-telemetry recordings of electroencephalogram, electrocardiogram, and breathing; (2) whole-body plethysmography; and (3) hypoglossal nerve recordings.
Results: In Atp1a3 mice, (1) SUDEP consistently occurred during seizures that were more severe than preterminal seizures; (2) seizure clustering occurred in periods preceding SUDEP; (3) slowing of breathing rate (BR) and heart rate was observed preictally before preterminal and terminal seizures; and (4) the sequence during terminal seizures was as follows: bradypnea with bradycardia/cardiac arrhythmias, then terminal apnea, followed by terminal cardiac arrhythmias.
Medicine (Baltimore)
January 2025
Department of Neonatal, Children's Hospital Affiliated to Shandong University/Jinan Children's Hospital, Jinan, Shandong, China.
Rationale: PCDH19-related epilepsy manifested various clinical features, including febrile epilepsy, with or without intellectual disability, and psych-behavioral disorders. However, there are few studies demonstrating abdominal pain as the first symptom.
Patient Concerns: A 3-year-old Chinese girl presented with clustered seizures of fever sensitivity accompanied by abdominal pain.
Brain Dev
January 2025
Department of Pediatrics, Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital, Seoul National University College of Medicine, 101 Daehakro, Jongno-Gu, Seoul 03080, Republic of Korea. Electronic address:
Objective: The present study aimed to investigate the initial clinical features of infantile-onset genetic epilepsy and compare initial seizure variables and responses to sodium channel blockers between SCN1A and non-SCN1A group.
Methods: We selected 122 patients, comprising 58 patients with SCN1A mutations and 64 patients with mutations in other than SCN1A, from our institutional database.
Results: Patients identified in the SCN1A group tended to present with fever, prolonged seizure duration, and hemiclonic seizure semiology.
J Neurodev Disord
January 2025
Graduate Neuroscience Program, University of California, Riverside, CA, USA.
Background: Fragile X syndrome (FXS) is a leading known genetic cause of intellectual disability and autism spectrum disorders (ASD)-associated behaviors. A consistent and debilitating phenotype of FXS is auditory hypersensitivity that may lead to delayed language and high anxiety. Consistent with findings in FXS human studies, the mouse model of FXS, the Fmr1 knock out (KO) mouse, shows auditory hypersensitivity and temporal processing deficits.
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