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Prognostic Value of Signal Abnormalities on Brain MRI in Post-Anoxic Super-Refractory Status Epilepticus: A Single-Center Retrospective Study.
Eur J Neurol
January 2025
Epilepsy Center, Department of Neurology, Fondazione IRCCS San Gerardo Dei Tintori, Monza, Italy.
Background: Epileptiform activity, including status epilepticus (SE), occurs in up to one-third of comatose survivors of cardiac arrest and may predict poor outcome. The relationship between SE and hypoxic-ischemic brain injury (HIBI) is not established.
Methods: This is a single-center retrospective study on consecutive patients with post-anoxic super-refractory SE.
Rare DDX3X Gene Mutation in a Male Newborn With Super-refractory Status Epilepticus Responding to Lacosamide Drug Therapy.
Cureus
December 2024
Pediatric Neurology, Bahrain Defence Force Hospital, Riffa, BHR.
Super-refractory status epilepticus (SRSE) is defined as status epilepticus that persists or recurs after treatment with anesthetic agents for more than 24 hours, including cases with recurrent seizures on reduction or withdrawal of anesthetic drugs. Super-refractory status epilepticus presents a significant challenge for neurologists, particularly when standard treatments fail to achieve seizure control. Lacosamide, which has a unique mechanism involving modulating voltage-gated sodium channels by enhancing their slow inactivation, has emerged as a potential option for managing SRSE.
View Article and Find Full Text PDFNORSE secondary to anti-GAD65 antibody-positive encephalitis treated with novel adjunctive rapid titration VNS protocol.
Epilepsia 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 PDFA Unique Case of MBD5 and CCM2 Deletions Leading to a Severe Neurological Phenotype With Prolonged Status Epilepticus.
Clin Genet
January 2025
Department of Human Genetics, Research Institute, National Center for Global Health and Medicine, Tokyo, Japan.
Heterozygous pathogenic variants in MBD5 (MIM*611472) and CCM2 (MIM*607929) cause autosomal dominant intellectual developmental disorder 1 (MIM#156200) and cerebral cavernous malformations-2 (MIM#603284), respectively. Both conditions may present with seizures, epilepsy, and status epilepticus. However, super-refractory status epilepticus, defined as seizures lasting more than 24 h, has not been described in either condition.
View Article and Find Full Text PDFQuantitative evaluation of electrographic response to electroconvulsive therapy in super-refractory status epilepticus.
Front Neurol
December 2024
Department of Neurology, University of California, San Francisco, San Francisco, CA, United States.
Objective: Electroconvulsive therapy (ECT) has been occasionally applied as a treatment for super-refractory status epilepticus (SRSE). However, the effects of ECT on electrographic activity and related clinical outcomes are largely unknown. Here, we use quantitative approaches on electroencephalography (EEG) data to evaluate the neurophysiological influences of ECT and how they may relate to patient survival.
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