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Children are particularly susceptible to the neurotoxic effects of organophosphates, which can lead to developmental neuronal deficits and associated dysfunction, including cognitive disabilities, epilepsy, and associated comorbidities. Anticonvulsants like benzodiazepines fail to prevent the lasting neurobehavioral and neuropathological effects of organophosphate exposure, emphasizing the need for new anticonvulsants to address these effects. This study evaluated the efficacy of the synthetic neurosteroid ganaxolone (GX) in combating persistent behavioral deficits, electrographic abnormalities, and neuropathological damage induced by diisopropylfluorophosphate (DFP) intoxication in pediatric rats. Postnatal day 21 rats were exposed to DFP acutely and were treated with GX (5-10 mg/kg). Behavior deficits were systematically monitored up to 3 months after exposure. Video electroencephalography at 3 months assessed spontaneous recurrent seizures, nonconvulsive epileptiform discharges, high-frequency oscillations, and interictal spike activity. GX treatment significantly mitigated anxiety, aggression, memory deficits, and depression-like phenotypes in DFP-exposed pediatric animals. It also reduced DFP-induced occurrence of epileptic biomarkers such as spontaneous recurrent seizures, epileptiform discharges, interictal spikes, and high-frequency oscillations demonstrating potential disease-modifying effects. Histological analysis showed that GX decreased the loss of parvalbumin (+) inhibitory neurons, neuronal nuclei antigen (+) principal neurons, and aberrant mossy fiber sprouting. GX also reduced neuroinflammation, indicated by decreased ionized calcium binding adaptor molecule 1 (+) microgliosis. Together, these results demonstrate the neuroprotective activity of GX in mitigating chronic neurologic dysfunction, neuroinflammation, and neurodegeneration and confirm GX as a promising treatment option for DFP exposure. SIGNIFICANCE STATEMENT: Acute organophosphate (OP) intoxication poses a severe risk, particularly to children, leading to life-threatening seizures and long-term neurological deficits. Current treatments, including benzodiazepines, are less effective against persistent seizures and neurological sequel after acute exposure. This study explores the potential of ganaxolone, a synthetic neurosteroid, to mitigate the neurodevelopmental consequences of OP exposure. Our findings reveal that ganaxolone provides significant neuroprotection in a pediatric model of OP intoxication, reducing long-term seizures, ictal biomarkers, neurodegeneration, neuroinflammation, and associated neurological dysfunctions, offering a promising therapeutic avenue for pediatric victims of OP exposure.
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http://dx.doi.org/10.1016/j.jpet.2025.103555 | DOI Listing |
J Neural Eng
September 2025
University of Pennsylvania, 3400 Spruce Street, Philadelphia, Pennsylvania, 19104-6243, UNITED STATES.
New implantable and wearable devices hold great promise to help patients manage their seizure disorders. One proposed application is measuring the rate of interictal epileptiform discharges as a biomarker of medication levels and seizure risk. This study aims to determine whether interictal epileptiform spike rates (spikes) are independently associated with anti-seizure medication (ASM) levels and evaluate whether spike rates are a reliable biomarker for ASM levels.
View Article and Find Full Text PDFJCI Insight
September 2025
Department of Physiology and Neurobiology, University of Connecticut, Storrs, United States of America.
Dravet syndrome (DS) is an early-onset epilepsy caused by loss of function mutations in the SCN1A gene, which encodes Nav1.1 channels that preferentially regulate activity of inhibitory neurons early in development. DS is associated with a high incidence of sudden unexpected death in epilepsy (SUDEP) by a mechanism that may involve respiratory failure.
View Article and Find Full Text PDFImmune Netw
August 2025
Department of Microbiology and Immunology, Yonsei University College of Medicine, Seoul 03722, Korea.
Developmental and epileptic encephalopathies (DEEs), including Infantile Epileptic Spasms Syndrome (IESS) and Lennox-Gastaut Syndrome (LGS), are severe pediatric conditions characterized by profound developmental delays and treatment-resistant epilepsy. Although steroid therapies provide some clinical benefits, the underlying immunological mechanisms remain poorly understood. In this study, we performed comprehensive immune profiling using multi-parametric flow cytometry on PBMCs from IESS (n=25) and LGS (n=9) patients, comparing them with age-matched healthy controls (n=54).
View Article and Find Full Text PDFFront Neurol
August 2025
Department of Neurosurgery, Haikou Hospital Affiliated with Xiangya Medical College, Central South University, Haikou, China.
As an emerging therapeutic strategy, stem cell transplantation has demonstrated promising potential in the management of refractory epilepsy. Epilepsy, a prevalent neurological disorder characterized by recurrent seizures, affects approximately one-third of patients worldwide who exhibit resistance to existing antiepileptic drugs (AEDs). Consequently, exploring novel treatment modalities is imperative.
View Article and Find Full Text PDFAm J Med Sci
September 2025
Department of Medicine, Division of Rheumatology, University of Oklahoma College of Medicine, Oklahoma City, OK; Department of Medicine, VAMC, Oklahoma City, OK. Electronic address:
Vagus nerve stimulation (VNS) has gained significant attention as a therapy for various medical conditions due to its ability to modulate chronic diseases, pain, and inflammation. VNS delivered by an implanted device is FDA approved for severe epilepsy and refractory depression. VNS delivered with implantable devices or transcutaneous methods are now being studied in several musculoskeletal diseases including osteoarthritis, rheumatoid arthritis, systemic lupus erythematosus, and fibromyalgia.
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