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Super refractory status epilepticus

Chih-Hsiang Lin, Yan-Ting Lu, Chen-Jui Ho, Fu-Yuan Shih, Meng-Han Tsai
Objective: The prognosis of status epilepticus (SE) is highly related to the underlying etiology. Inflammation of the central nervous system (CNS), including infection and autoimmune encephalitis, is one of the treatable conditions causing SE. The initial presentation of infectious and autoimmune CNS disorders can be quite similar, which may be difficult to differentiate at the beginning. However, treatment for these entities can be quite different. In this study, we aim to identify the differences in clinical features among patients with infectious and autoimmune SE, which could help the clinicians to select initial investigation and ensuing therapies that may improve overall outcomes...
2019: Frontiers in Neurology
Hea Ree Park, Pamela Song, Jae Jung Lee, Joong-Yang Cho
Endosulfan is a highly toxic pesticide that causes hyperstimulation of the central nervous system by antagonizing gamma aminobutyric acid-mediated inhibition. Seizure is the most important manifestation of endosulfan poisoning, frequently progressing to status epilepticus and refractory status epilepticus. Here, we report a recent case of a 64-year-old man with endosulfan-induced super-refractory status epilepticus, which persisted for a remarkably longer period than has been described in previous reports. The patient arrived at the emergency room with continuous generalized tonic-clonic seizures...
December 2018: Journal of Epilepsy Research
Mohankumar Kurukumbi, James Leiphart, Anam Asif, Jing Wang
The treatment protocol of status epilepticus has many associated toxicities so there is interest in alternate nonmedicinal therapies for managing New Onset Refractory Status Epilepticus (NORSE) patients. Vagus nerve stimulation (VNS) is an FDA-approved therapy for refractory epilepsy that has been shown to decrease the frequency and severity of seizures. We present the case of a patient with new-onset refractory status epilepticus (NORSE) whose seizures were successfully treated with vagus nerve stimulation...
2019: Case Reports in Neurological Medicine
Salia Farrokh, Michael Erdman, John Bon, Eljim Tesoro
Status Epilepticus (SE) has a high mortality rate and is one of the most common neurological emergencies. Fast progression of this neurological emergency and lack of response to traditional antiepileptic drugs (AEDs) in most cases has challenged clinicians to use new agents. The objective of this paper was to evaluate the efficacy and safety of AEDs released to the market after 2000 for SE, refractory SE (RSE), and super refractory SE (SRSE). The PubMed database was searched for clinical trials published between January 2000 and July 2018 using the search terms status epilepticus, refractory status epilepticus, super refractory status epilepticus, brivaracetam, clobazam, cannabidiol, eslicarbazepine, lacosamide, perampanel, rufinamide, stiripentol, and zonisamide...
February 5, 2019: Pharmacotherapy
Daniel San-Juan, Luis Ángel Álvarez-Perera, Daniel Oswaldo Dávila-Rodríguez, Christian Ramos-Jiménez, Víctor Alcocer-Barrada, Martha Lilia-Tena, David J Anschel, Jocelyn Pérez Cruz, Iris Enriqueta Martínez-Juárez
BACKGROUND: Super refractory epilepticus status (SRSE) is a life-threatening neurologic emergency defined as 'status epilepticus (SE) that continues 24 hours or more after the onset of anaesthesia, including those cases in which the SE recurs on the reduction or withdrawal of anaesthesia', which occur in 10-15% of SE patients and rarely has been resolved surgically. METHODS: A 20-year-old man with SRSE and a long history of left parieto-occipital oligoastrocytoma was admitted for convulsive SE that become SRSE and underwent lesionectomy guided by electrocorticography and neuro-navigation for local tumor recurrence...
January 22, 2019: World Neurosurgery
Bandya Sahoo, Mukesh Kumar Jain, Reshmi Mishra, Sibabratta Patnaik
Objective: The main objective is to assess the challenges in diagnosis and treatment while managing seronegative cases of autoimmune encephalitis (AIE) in Indian children. Methods: A cohort study of patients with AIE was done where clinical presentations, investigations, management were analyzed and these patients were followed up to assess the evolution of the disease. Results: Nine patients were included in the study. Four patients presented with super-refractory status epilepticus (SRSE)...
December 2018: Indian Journal of Critical Care Medicine
Eu Gene Park, Jiwon Lee, Jeehun Lee
OBJECTIVES: Super-refractory status epilepticus (SRSE) is one of the most challenging issues in intensive care units (ICUs) in that it is associated with high morbidity and mortality. Although the ketogenic diet (KD) has been reported to be effective in treating of SRSE, the use of the diet as therapy can be complicated by concomitant medical problems specific to critically ill patients. In this study, we aimed to describe our experience of the KD for SRSE patients in ICUs. METHODS: We retrospectively reviewed the medical records of 16 patients (10 males, 6 females) with SRSE who were treated with the KD in the ICUs at Samsung Medical Center from July 2005 to July 2017...
January 9, 2019: Brain & Development
Vasiliki Pantazou, Jan Novy, Andrea O Rossetti
INTRODUCTION: Status epilepticus (SE) represents a neurological emergency that leads to considerable morbidity and mortality. Following failure of first-line therapy, usually with benzodiazepines, there is no clear evidence to guide treatment of refractory SE, although a wide variety of approaches has been described anecdotally. OBJECTIVE: The aim of this study was to assess the clinical response to corticosteroids in adults with refractory and super-refractory SE, describing, to the best of our knowledge, the first adult SE cohort treated with corticosteroids...
January 9, 2019: CNS Drugs
Adam Strzelczyk, Susanne Knake, Reetta Kälviäinen, Estevo Santamarina, Manuel Toledo, Sophia Willig, Alexandra Rohracher, Eugen Trinka, Felix Rosenow
OBJECTIVE: Novel treatments are needed to control treatment-resistant status epilepticus (SE). We report a summary of clinical cases where perampanel was used in established SE, refractory SE (RSE), or super-refractory SE (SRSE). METHODS: Medical records were retrospectively reviewed for perampanel administration in SE at five European hospitals between 2011 and 2015. RESULTS: Of 1319 patients identified as experiencing SE, 52 (3.9%) received perampanel...
January 4, 2019: Acta Neurologica Scandinavica
Jaideep Kapur
This review considers the role of N -methyl-d-aspartate receptors in the pathophysiology and treatment of status epilepticus (SE). NMDA receptors play a critical role in sustaining SE by mediating the plasticity of γ-aminobutyric acid (GABA)-A and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptors, neuronal loss, and epileptogenesis. In parallel, there is growing interest in using the NMDA receptor antagonist ketamine in the treatment of refractory SE. Ketamine has proved to be safe for use in refractory and super-refractory SE in patients...
December 2018: Epilepsia Open
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
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