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Super refractory status epilepticus

Lia D Ernst, Katie L Krause, Marissa A Kellogg, Ahmed M Raslan, David C Spencer
There are very few randomized controlled trials studying treatment of super refractory status epilepticus (SE), despite estimated occurrence in about 15% of SE cases and its association with high morbidity and mortality rates. Small case series and case reports have described use of neurostimulation, including vagal nerve stimulation, transcranial magnetic stimulation, and deep brain stimulation, to treat super refractory SE when medical interventions have failed. To our knowledge, this is the first reported case of responsive neurostimulation being used to successfully treat a case of super refractory SE...
December 6, 2018: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
Palita Arayakarnkul, Krisnachai Chomtho
BACKGROUND: Super-refractory status epilepticus (SRSE) is a seizure that continues >24 h after anesthesia, or recurs on the reduction of anesthesia. SRSE is extremely difficult-to-control and associated with poor outcome. To date, optimal therapy and outcome data in children is limited. OBJECTIVE: To assess etiology, treatment options and outcome in pediatric SRSE patients. METHOD: We reviewed medical records of children <15 years old with SRSE during 2007-2017 at King Chulalongkorn Memorial Hospital...
December 7, 2018: Brain & Development
Sarah E Nelson, Panayiotis N Varelas
PURPOSE OF REVIEW: Status epilepticus, refractory status epilepticus, and super-refractory status epilepticus can be life-threatening conditions. This article presents an overview of the three conditions and discusses their management and outcomes. RECENT FINDINGS: Status epilepticus was previously defined as lasting for 30 minutes or longer but now is more often defined as lasting 5 minutes or longer. A variety of potential causes exist for status epilepticus, refractory status epilepticus, and super-refractory status epilepticus, but all three ultimately involve changes at the cellular and molecular level...
December 2018: Continuum: Lifelong Learning in Neurology
Tatiana Potes, Santiago Galicchio, Bárbara Rosso, Gabriela Besocke, María Del Carmen García, Juan Carlos Avalos
Lafora's disease is infrequent. However, it is one of the most common causes of progressive myoclonus epilepsy. We present the case of a 19-year-old woman, without comorbidities and normal development that started at 8 years with seizures and that from 15 years, had progressive cognitive deterioration. She was admitted to our institution with a diagnosis of super refractory status epilepticus. The diagnosis of Lafora's disease was made through pathological anatomy, later a genetic test was performed that reported a pathogenic variant of the EPM2A gene, confirming the diagnosis...
2018: Medicina
Brandon A Francis, Jennifer Fillenworth, Philip Gorelick, Kristina Karanec, Adriana Tanner
BACKGROUND: Status, refractory status and super refractory status epilepticus are common neurologic emergencies. The objective of this study is to investigate the feasibility, safety and effectiveness of a ketogenic diet (KD) for refractory status epilepticus (RSE) in adults in the intensive care unit (ICU). METHODS: We performed a retrospective, single-center study of patients between ages 18 and 80 years with RSE treated with a KD treatment algorithm from November 2016 through April 2018...
November 27, 2018: Neurocritical Care
Claudine Sculier, Nicolas Gaspard
PURPOSE: To summarize the clinical features, suggested work-up, treatment and prognosis of new-onset refractory status epilepticus (NORSE), a condition recently defined as the occurrence of refractory status epilepticus (RSE) in a patient without active epilepsy, and without a clear acute or active structural, toxic or metabolic cause; and of the related syndrome of febrile infection-related epilepsy syndrome (FIRES), also recently defined as a subgroup of NORSE preceded by a febrile illness between 2 weeks and 24 h prior to the onset of RSE...
September 29, 2018: Seizure: the Journal of the British Epilepsy Association
Dominik Madžar, Caroline Reindl, Antje Giede-Jeppe, Tobias Bobinger, Maximilian I Sprügel, Ruben U Knappe, Hajo M Hamer, Hagen B Huttner
BACKGROUND: Patients in refractory status epilepticus (RSE) may require treatment with continuous intravenous anesthetic drugs (cIVADs) for seizure control. The use of cIVADs, however, was recently associated with poor outcome in status epilepticus (SE), raising the question of whether cIVAD therapy should be delayed for attempts to halt seizures with repeated non-anesthetic antiepileptic drugs. In this study, we aimed to determine the impact of differences in therapeutic approaches on RSE outcome using timing of cIVAD therapy as a surrogate for treatment aggressiveness...
November 21, 2018: Critical Care: the Official Journal of the Critical Care Forum
Daniel Gomes, José Pimentel, Carla Bentes, Diana Aguiar de Sousa, Ana Patrícia Antunes, António Alvarez, Zélia Costa Silva
INTRODUCTION: Super-refractory status epilepticus is defined as status epilepticus that persists or recurs 24 hours after anaesthetic therapy onset or after its withdrawal. It is mostly found in intensive care units and carries high mortality but good long-term prognosis for those who survive. In contrast with the initial phases of status epilepticus, treatment lacks strong scientific evidence and is mostly derived from case reports or small case series. OBJECTIVE: To propose a protocol for the treatment of super-refractory status epilepticus in level III intensive care units, focusing on the treatment strategies to control clinical and/or electroencephalographic epileptic activity...
October 31, 2018: Acta Médica Portuguesa
Fang Yuan, Fang Yang, Ruihua Jia, Wen Li, Yongli Jiang, Jingjing Zhao, Wen Jiang
Objective: Status epilepticus (SE) is one of the most critical symptoms of encephalitis. Studies on early predictions of progression to super-refractory status epilepticus (SRSE) and poor outcome in SE due to acute encephalitis are scarce. We aimed to investigate the values of neuroimaging and continuous electroencephalogram (EEG) in the multimodal prediction. Methods: Consecutive patients with convulsive SE due to acute encephalitis were included in this study. Demographics, clinical features, neuro-imaging characteristics, medical interventions, and anti-epileptic treatment responses were collected...
2018: Frontiers in Neurology
Baoqiong Liu, Yan Zhou, Lingbin Meng, Holly Skinner
Limbic encephalitis (LE) is a neurological syndrome that mainly affects mesial temporal lobes. It may present in association with cancer or infection. Limbic encephalitis associated with glutamic acid decarboxylase antibodies (anti-GAD) is rare. Here, we report a case of anti-GAD limbic encephalitis to heighten the awareness of this rare cause of autoimmune encephalitis. Anti-GAD-associated epilepsy is often poorly responsive to seizure medications. Treatment is challenging. Early initiation of immunotherapy is important...
August 10, 2018: Curēus
Ayush Agarwal, Deepti Vibha, Rohan Chawla, Mehar Chand Sharma
We present a case of a 23-year-old man with history of fever followed by painless complete vision loss, with subsequent new-onset refractory status epilepticus (NORSE). He initially developed bilateral retinitis. A few days later, he started having focal seizures, and subsequently developed super-refractory status epilepticus, requiring anaesthetic agents. MRI brain revealed multifocal cortical and subcortical hyperintensities in occipital and temporoparietal regions without contrast enhancement. MRI repeated a month later showed new lesions with non-visualisation of some previous lesions...
October 12, 2018: BMJ Case Reports
Ravindra Arya, Alexander Rotenberg
PURPOSE: To summarize the evidence regarding dietary, immunological, surgical, and other emerging treatments for refractory status epilepticus (RSE)/super-RSE (SRSE). METHODS: Narrative literature review including relevant human studies. RESULTS: Hypothermia and brenaxolone were tested in randomized controlled trials for RSE/SRSE management, while other interventions have only limited evidence for their efficacy and safety. Clinical trials including the HYBERNATUS study found the efficacy of therapeutic hypothermia to be no better than placebo for RSE/SRSE, and raised concerns about its safety...
September 14, 2018: Seizure: the Journal of the British Epilepsy Association
María Celeste Buompadre
Status epilepticus (SE) is one of the most common neurologic emergencies in pediatrics. It is a condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which leads to abnormally, prolonged seizures. This definition provides a good guidance, when emergency treatment must be considered. In general, time point t1 is the time when treatment should be started, which is at 5 minutes for generalized tonic-clonic seizures, and at 10 min for focal seizures with or without impairment of consciousness...
2018: Medicina
Iván Sánchez Fernández, Tobias Loddenkemper
PURPOSE: To estimate the cost of admissions related to status epilepticus (SE) in the USA and to evaluate SE mortality. METHOD: Descriptive retrospective study using national estimates from the KID's Inpatient Database (KID) for children and from the National Inpatient Sample (NIS) for adults for the years 2007-2012, the largest collection of all-payer, encounter-level hospital care data in the United States. The individual observation in this study is hospital admission...
October 2018: Seizure: the Journal of the British Epilepsy Association
Monica Ferlisi, Sara Hocker, Eugen Trinka, Simon Shorvon
To describe the demographics, etiologies, types of status epilepticus (SE), and outcomes in people with refractory and super-refractory SE from around the world, we prospectively collected cases of refractory SE (RSE) treated with continuous intravenous anesthetic drugs in an intensive care unit setting through online questionnaires using "active surveillance." We collected information about 776 cases of RSE in 50 countries over 4 years. Control of SE was achieved in 74% of the cases. Neurologic outcomes were poor in 41% of patients, and 24% died...
October 2018: Epilepsia
Simone Beretta, Giada Padovano, Andrea Stabile, Anna Coppo, Graziella Bogliun, Leonello Avalli, Carlo Ferrarese
Refractory nonconvulsive status epilepticus (NCSE) occurs in 10%-30% of patients following resuscitation after cardiac arrest. Both the optimal treatment and prognosis of postanoxic status epilepticus remain uncertain. We analyzed acute electroencephalographic changes, neurological outcome at 3 months, and adverse effects in consecutive postanoxic patients with super-refractory NCSE treated with add-on oral loading of perampanel. Eight postanoxic patients with super-refractory NCSE were treated with perampanel (dose range = 6-12 mg)...
October 2018: Epilepsia
Andrea O Rossetti
The treatment of refractory or super-refractory status epilepticus (SE) currently relies on low-evidence strategies, including general anesthetics to induce pharmacologic coma, ketogenic diet, immunosuppression, and other physical measures. Besides the formal uncertainty regarding efficacy, concerns have been about tolerability. In this situation, identification of alternative, higher evidence treatments is urgently needed. Allopregnanolone is an endogenous neurosteroid exerting a positive allosteric modulation on γ-aminobutyric acid (GABA)A receptors...
October 2018: Epilepsia
Daniele Grioni, Andrea Landi, Leonardo Fiori, Erik Pietro Sganzerla
PURPOSE: Status Epilepticus can be a serious life threatening event in epileptic patients. The definition of refractory or super-refractory Status Epilepticus was based on the therapeutic response to anti-epileptic and anesthetic drugs. Vagal Nerve Stimulation showed efficacy in treating drug-resistant epilepsy but there are only few reports on emergentplacement of Vagal Nerve Stimulator for refractory or super-refractory Status Epilepticus. METHODS: Among 49 children implanted at our Institution with Vagal Nerve Stimulation for drug-resistant epilepsy, the authors retrospectively identified those implanted for refractory or super-refractory Status Epilepticus, according with the current definitions...
October 2018: Seizure: the Journal of the British Epilepsy Association
Francesco Brigo, Simona Lattanzi, Alexandra Rohracher, Emilio Russo, Stefano Meletti, Elisabetta Grillo, Eugen Trinka
OBJECTIVE: Perampanel (PER) is a noncompetitive β-amino-3-(5-methyl-3-oxo-1,2-oxazol-4-yl)propionic acid (AMPA) receptor antagonist with demonstrated efficacy in animal models of status epilepticus (SE). We performed a systematic review of the literature to assess the efficacy and tolerability of PER in the treatment of refractory and super-refractory SE. METHODS: We searched Medline, Embase, and CENTRAL (accessed from inception to April 30, 2018) to identify studies evaluating oral PER as treatment of SE of any type...
September 2018: Epilepsy & Behavior: E&B
Joseph R Blunck, Joseph W Newman, Ronald K Fields, John E Croom
Background: A ketogenic diet (KD) may have a role in treating patients in super-refractory status epilepticus (SRSE). Sodium-glucose cotransporter 2 (SGLT2) inhibitors have a risk of ketoacidosis that could facilitate induction of KD. Case summary: A 42-year-old with a history of drug resistant epilepsy developed SRSE requiring several pharmacological interventions during her hospital course including the initiation of KD that failed. SGLT2 inhibitor therapy was initiated in a successful attempt to augment ketone production...
2018: Epilepsy & Behavior Case Reports
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