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Revised Process for ACNS Guidelines Development.
J Clin Neurophysiol
February 2025
Division of Child Neurology, Department of Neurology, Stanford University, Palo Alto, California, U.S.A.
The development of clinical practice guidelines is an evolving field. In response to the need for consistent, evidence-based medical practice, the American Clinical Neurophysiology Society identified the need to update the Society's guideline development process. The American Clinical Neurophysiology Society Guidelines Committee created an action plan with the goal of improving transparency and rigor for future guidelines and bringing existing guidelines to current standards.
View Article and Find Full Text PDFDownmodulation of potassium conductances induces epileptic seizures in cortical network models via multiple synergistic factors.
J Neurosci
January 2025
Department of Neuroscience, Brown University, Providence RI, USA.
Voltage-gated potassium conductances [Formula: see text] play a critical role not only in normal neural function, but also in many neurological disorders and related therapeutic interventions. In particular, in an important animal model of epileptic seizures, 4-aminopyridine (4-AP) administration is thought to induce seizures by reducing [Formula: see text] in cortex and other brain areas. Interestingly, 4-AP has also been useful in the treatment of neurological disorders such as multiple sclerosis (MS) and spinal cord injury, where it is thought to improve action potential propagation in axonal fibers.
View Article and Find Full Text PDFAdaptive protein synthesis in genetic models of copper deficiency and childhood neurodegeneration.
Mol Biol Cell
January 2025
Department of Cell Biology, Emory University, 615 Michael St, Atlanta, GA, USA, 30322.
Rare inherited diseases caused by mutations in the copper transporters (CTR1) or induce copper deficiency in the brain, causing seizures and neurodegeneration in infancy through poorly understood mechanisms. Here, we used multiple model systems to characterize the molecular mechanisms by which neuronal cells respond to copper deficiency. Targeted deletion of CTR1 in neuroblastoma cells produced copper deficiency that produced a metabolic shift favoring glycolysis over oxidative phosphorylation.
View Article and Find Full Text PDFHuman TSC2 mutant cells exhibit aberrations in early neurodevelopment accompanied by changes in the DNA Methylome.
Hum Mol Genet
January 2025
Department of Cell & Developmental Biology, Vanderbilt University School of Medicine, 1161 21st Ave S, Nashville, Tennessee, 37232, United States of America.
Tuberous Sclerosis Complex (TSC) is a debilitating developmental disorder characterized by a variety of clinical manifestations. While benign tumors in the heart, lungs, kidney, and brain are all hallmarks of the disease, the most severe symptoms of TSC are often neurological, including seizures, autism, psychiatric disorders, and intellectual disabilities. TSC is caused by loss of function mutations in the TSC1 or TSC2 genes and consequent dysregulation of signaling via mechanistic Target of Rapamycin Complex 1 (mTORC1).
View Article and Find Full Text PDFImmediate Postoperative Activation of Vagus Nerve Stimulation (VNS) for Super-refractory Status Epilepticus: A Case Report.
Cureus
December 2024
Clinical Research, National Institute of Neurology and Neurosurgery, Mexico City, MEX.
Anti-NMDA (N-methyl-D-aspartate) receptor encephalitis (ANRE) is a rare autoimmune condition targeting brain receptors, often linked to ovarian tumors in young women. In severe cases, it can lead to status epilepticus, but in sporadic cases, it may progress to super-refractory status epilepticus (SRSE), a dangerous state of continuous or repetitive seizures demanding urgent medical attention that continues or recurs more than 24 hours after the initiation of anesthetic therapy. We present a case report of anti-NMDA receptor limbic encephalitis-triggered SRSE terminated with vagus nerve stimulation (VNS) and titrated to high stimulation parameters in the immediate postoperative period.
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