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Up to What Extent Does Dravet Syndrome Benefit From Neurostimulation Techniques?

FRONTIERS IN NEUROLOGY (2022)

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Short-term transcutaneous non-invasive vagus nerve stimulation may reduce disease activity and pro-inflammatory cytokines in rheumatoid arthritis: results of a pilot study

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Summary: In patients with active rheumatoid arthritis, transcutaneous non-invasive vagus nerve stimulation (n-VNS) showed anti-inflammatory effects by reducing disease activity scores, C-reactive protein levels, and interferon-gamma. However, in patients with inactive disease, n-VNS did not affect disease activity scores, but led to a decrease in cardiac vagal tone and interleukin-10 levels.

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Summary: Dravet syndrome is a severe form of epilepsy that starts in infancy, characterized by seizures triggered by high fever. It has a progression of worsening and stabilization stages, with most patients having a mutation in the SCN1A gene. Current treatment options include various drugs under clinical evaluation for potential efficacy.

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Summary: In this meta-analysis, it was found that corpus callosotomy (CC) appeared to be more effective than vagus nerve stimulation (VNS) for reducing seizures. VNS may provide similar or higher levels of quality of life improvement with lower aggregate risk of complications. Patient selection, anatomy, and seizure type are important factors in decision-making.

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Summary: After electrode implantation, a reduction in seizure frequency was observed in patients with focal epilepsy before stimulation initiation, suggesting the occurrence of an insertional effect (IE). The reduction in seizure frequency was found to be independent of the number of implanted electrodes and the stimulation target, with the peak of the lesional effect occurring at two to three months after electrode insertion in a subgroup of epilepsy patients.

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Two-center experience of cannabidiol use in adults with Dravet syndrome

Katri Silvennoinen et al.

Summary: In two UK neurology centres, 18 adults with Dravet Syndrome were treated with cannabidiol (CBD), with some experiencing a significant reduction in convulsive seizures, although a relatively high rate of adverse events was observed.

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SCN1A Mutation-Beyond Dravet Syndrome: A Systematic Review and Narrative Synthesis

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Summary: SCN1A mutations are not only associated with Dravet syndrome, but also with various other epileptic encephalopathies and related disorders.

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Network Substrates of Centromedian Nucleus Deep Brain Stimulation in Generalized Pharmacoresistant Epilepsy

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Time to onset of cannabidiol treatment effects in Dravet syndrome: Analysis from two randomized controlled trials

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Improved everyday executive functioning following profound reduction in seizure frequency with fenfluramine: Analysis from a phase 3 long-term extension study in children/young adults with Dravet syndrome

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Summary: In children and young adults with DS, reduction in seizure frequency after long-term treatment with adjunctive fenfluramine was associated with clinically meaningful improvements in everyday executive functions. 78% of children and young adults achieved a >= 50% reduction in seizure frequency, which was significantly associated with greater likelihood of clinically meaningful improvement in emotion regulation and cognitive regulation.

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Long-term results of vagus nerve stimulation in children with Dravet syndrome: Time-dependent, delayed antiepileptic effect

Song Ee Youn et al.

Summary: The study aimed to evaluate the long-term outcomes of vagus nerve stimulation (VNS) in children with pharmaco-resistant Dravet syndrome (DS). Results showed that VNS therapy led to long-term, progressive, and time-dependent improvement in seizure control for children with pharmaco-resistant DS.

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Is vagal-nerve stimulation safe during pregnancy? A mini review

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Dose-Ranging Effect of Adjunctive Oral Cannabidiol vs Placebo on Convulsive Seizure Frequency in Dravet Syndrome A Randomized Clinical Trial

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Hippocampal deletion of NaV1.1 channels in mice causes thermal seizures and cognitive deficit characteristic of Dravet Syndrome

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