The development and initial validity and reliability testing of a single-item, 7-point global rating scale designed for neurologists to assess the overall severity of epilepsy in children, the Global Assessment of Severity of Epilepsy (GASE) Scale, is described. The GASE Scale was quick and easy to use. Median epilepsy severity in the development sample was 3 (moderately severe), with a range from 1 ("not severe at all") in 36 patients (26.9%) to 7 ("extremely severe") in 7 patients (5.2%). Preliminary evidence of construct validity was found in support for our a priori predictions of associations between GASE scores and neurologists' ratings of seven individual clinical aspects of epilepsy and in a cumulative R(2) for the GASE score of 81% using ratings of the clinical aspects of epilepsy. Weighted kappa was 0.85 (95% CI: 0.79, 0.90) for inter-rater reliability and 0.90 (95% CI: 0.82, 0.98) and 0.95 (95% CI: 0.91, 0.98) for test-retest reliability for each of two raters. These promising initial results support continuation of the multistage process of testing the validity and reliability of the GASE Scale within various clinical contexts.
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http://dx.doi.org/10.1016/j.yebeh.2008.05.001 | DOI Listing |
Epilepsia
January 2025
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Objective: To evaluate iron deposition patterns in patients with cerebral cavernous malformation-related epilepsy (CRE) using quantitative susceptibility mapping (QSM) for detailed analysis of iron distribution associated with a history of epilepsy and severity.
Methods: This study is part of the Quantitative Susceptibility Biomarker and Brain Structural Property for Cerebral Cavernous Malformation Related Epilepsy (CRESS) cohort, a prospective multicenter study. QSM was used to quantify iron deposition in patients with sporadic cerebral cavernous malformation (CCMs).
Neuronal pentraxin 2 (NP2) plays a significant role in synaptic plasticity, neuronal survival, and excitatory synapse regulation. Emerging research suggests that NP2 is implicated in the pathogenesis of various neurological disorders, including neurodegenerative diseases, neuropsychiatric disorders, and neuropathies. This literature review extensively analyzes NP2's role in these conditions, thereby highlighting its contributions to synaptic dysfunction, neuroinflammation, and neurotoxic protein aggregation.
View Article and Find Full Text PDFEpilepsia Open
January 2025
Neurology Department, Wellstar MCG Health at the Medical College of Georgia, Augusta, Georgia.
New Onset Refractory Status Epilepticus (NORSE) is a rare and severe condition characterized by refractory seizures in individuals without a prior history of epilepsy. This case report describes a 37-year-old woman diagnosed with anti-glutamic acid decarboxylase 65 (anti-GAD65) antibody-positive encephalitis-related NORSE. Her seizures were refractory to multiple interventions, including anti-seizure medications, anesthetics, immunotherapies, a ketogenic diet, and electroconvulsive therapy.
View Article and Find Full Text PDFNeuropharmacology
January 2025
Pharmacology and Toxicology Department, Faculty of Pharmacy, Cairo University, Cairo, Egypt; Pharmacology and Toxicology Department, Faculty of Pharmacy, King Salman International University (KSIU), South Sinai 46612, Egypt.
Seizures can lead to cardiac dysfunction. Multiple pathways contribute to this phenomenon, of which the chaperone sigma-1 receptor (S1R) signaling represents a promising nexus between the abnormalities seen in both epilepsy and ensuing cardiac complications. The study explored the potential of Berberine (BER), a promising S1R agonist, in treating epilepsy and associated cardiac abnormalities in a pentylenetetrazol (PTZ) kindling rat model of epilepsy.
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.
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