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Super refractory status epilepticus

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...
August 11, 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...
July 31, 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
Alexandra Rohracher, Gudrun Kalss, Caroline Neuray, Julia Höfler, Judith Dobesberger, Giorgi Kuchukhidze, Rudolf Kreidenhuber, Cristina Florea, Aljoscha Thomschewski, Helmut F Novak, Georg Pilz, Markus Leitinger, Eugen Trinka
In refractory status epilepticus (SE), γ-aminobutyric acidergic drugs become less effective and glutamate plays a major role in seizure perpetuation. Data on the efficacy of perampanel (PER) in treatment of refractory SE in humans are limited. Here, we present a single-center case series of patients with refractory SE who received PER orally in an intensive care unit. We retrospectively analyzed treatment response, outcome, and adverse effects of all patients with refractory SE in our Neurological Intensive Care Unit who received add-on PER between September 2012 and February 2018...
July 25, 2018: Epilepsia
Wen-Yu Lu, Wen-Chin Weng, Lee-Chin Wong, Wang-Tso Lee
BACKGROUND: Both refractory convulsive status epilepticus (SE) and super-refractory SE are medical emergencies. However, there are limited data on super-refractory SE in children. Thus, this study focuses on characterizing the demographics, outcomes, and prognostic factors for super-refractory SE in children. METHODS: This study was a retrospective analysis of super-refractory SE treated in a tertiary referral center in Taiwan. The functional outcome was evaluated by modified Rankin scale (mRS)...
July 10, 2018: Epilepsy & Behavior: E&B
Diane C McLaughlin, Jorge M Mallea, Lauren K Ng
Hyperammonemia is a rare complication of lung transplant with a high mortality rate. It presents as encephalopathy and progresses to seizures, status epilepticus, coma, cerebral edema, and brain death. Multiple treatments have been documented including administration of medications, gut decontamination, and dialysis. However, no definitive treatments exist and mortality remains between 67% and 75%. We present the case of a 65-year-old male with idiopathic pulmonary fibrosis who developed refractory status epilepticus secondary to hyperammonemia following lung transplant...
June 2018: Indian Journal of Critical Care Medicine
Dheeraj Masapu, K N Gopala Krishna, Sinha Sanjib, Dhrithiman Chakrabarti, R C Mundlamuri, Nitin Manohar, P Mariamma, P Satishchandra, G S Umamaheswara Rao
Background: The recommended treatment for refractory status epilepticus (RSE) is the use of anesthetic agents, but evidence regarding the agent of choice is lacking. This study was designed to compare target-controlled infusion of propofol versus midazolam for the treatment of RSE regarding seizure control and complications. Methods: This prospective, randomized study recruited 23 adult patients with RSE due to any etiology and treated with either propofol or midazolam titrated to clinical seizure cessation and gradual tapering thereafter...
June 2018: Indian Journal of Critical Care Medicine
Alvin S Das, Jong Woo Lee, Eric S Rosenthal, Henrikas Vaitkevicius
BACKGROUND: Management of refractory status epilepticus (SE) commonly involves the induction of burst suppression using intravenous anesthetic agents. However, the endpoints of these therapies are not well defined. Weaning anesthetic agents are complicated by the emergence of electroencephalogram (EEG) patterns along the ictal-interictal continuum (IIC), which have uncertain significance given that IIC patterns may worsen cerebral metabolism and oxygenation, have a dissociation between scalp and depth EEG recordings, or may indicate a late stage of SE itself...
June 8, 2018: Neurocritical Care
Alejandra Vasquez, Raquel Farias-Moeller, William Tatum
PURPOSE: To summarize the available evidence related to pediatric refractory status epilepticus (RSE) and super-refractory status epilepticus (SRSE), with emphasis on epidemiology, etiologies, therapeutic approaches, and clinical outcomes. METHODS: Narrative review of the medical literature using MEDLINE database. RESULTS: RSE is defined as status epilepticus (SE) that fails to respond to adequately used first- and second-line antiepileptic drugs...
May 19, 2018: Seizure: the Journal of the British Epilepsy Association
E Ly Chan, W C Lee, C K Koo, H St King, C T Woo, S H Ng
No abstract text is available yet for this article.
June 2018: Hong Kong Medical Journal, Xianggang Yi Xue za Zhi
Samhitha Rai, Frank W Drislane
Refractory and super-refractory status epilepticus (SE) are serious illnesses with a high risk of morbidity and even fatality. In the setting of refractory generalized convulsive SE (GCSE), there is ample justification to use continuous infusions of highly sedating medications-usually midazolam, pentobarbital, or propofol. Each of these medications has advantages and disadvantages, and the particulars of their use remain controversial. Continuous EEG monitoring is crucial in guiding the management of these critically ill patients: in diagnosis, in detecting relapse, and in adjusting medications...
June 19, 2018: Neurotherapeutics: the Journal of the American Society for Experimental NeuroTherapeutics
Conor S Ryan, Anthony L Fine, Alexander L Cohen, Brenda M Schiltz, Deborah L Renaud, Elaine C Wirrell, Marc C Patterson, Nicole J Boczek, Raymond Liu, Dusica Babovic-Vuksanovic, David C Chan, Eric T Payne
BACKGROUND: The dynamin 1-like gene ( DNM1L) encodes a GTPase that mediates mitochondrial and peroxisomal fission and fusion. We report a new clinical presentation associated with a DNM1L pathogenic variant and review the literature. RESULTS: A 13-year-old boy with mild developmental delays and paroxysmal dystonia presented acutely with multifocal myoclonic super-refractory status epilepticus. Despite sustained and aggressive treatment, seizures persisted and care was ultimately withdrawn in the context of extensive global cortical atrophy...
January 1, 2018: Journal of Child Neurology
Jennifer Erklauer, Jeanine Graf, Mona McPherson, Anne Anderson, Angus Wilfong, Charles G Minard, Laura Loftis
BACKGROUND: Functional neurologic outcome for children with refractory and super-refractory status epilepticus has not been well defined. METHODS: Retrospective chart review including children age 0-17 years who received pentobarbital infusion from 2003 to 2016 for status epilepticus. Outcomes were defined in terms of mortality, need for new medical technology assistance at hospital discharge and functional neurologic outcome determined by pediatric cerebral performance category score (PCPC)...
March 26, 2018: Neurocritical Care
P Peng, X L Deng, L F Yang
No abstract text is available yet for this article.
March 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Sandra Pereira, Mariana Adrião, Mafalda Sampaio, Margarida Ayres Basto, Esmeralda Rodrigues, Laura Vilarinho, Elisa Leão Teles, Isabel Alonso, Miguel Leão
INTRODUCTION: Combined oxidative phosphorylation deficiency 20 (COXPD20) is a mitochondrial respiratory chain complex (RC) disorder, caused by disease-causing variants in the VARS2 gene, which encodes a mitochondrial aminoacyl-tRNA synthetase. Here we describe a patient with fatal mitochondrial encephalopathy caused by a homozygous VARS2 gene missense variant. CASE REPORT: We report the case of a girl, the first child of non-consanguineous and healthy parents, born from an uneventful term pregnancy, who presented, in the neonatal period, major hypotonia and microcephaly...
February 25, 2018: JIMD Reports
Dnyaneshwar P Mutkule, S Manimala Rao, Jaydip Ray Chaudhuri, Kunche Rajasri
Status epilepticus is frequently encountered in neuro Intensive Care Units. It is a medical emergency and if not treated promptly can lead to severe brain damage and even death. Here, we present the case of a 18-year-old male with uncontrolled and unrelenting seizures with a rare etiology requiring ketamine infusion for burst suppression as it was resistant to thiopentone and midazolam infusions. The management of this case is presented in detail.
January 2018: Indian Journal of Critical Care Medicine
Lawrence J Hirsch, Nicolas Gaspard, Andreas van Baalen, Rima Nabbout, Sophie Demeret, Tobias Loddenkemper, Vincent Navarro, Nicola Specchio, Lieven Lagae, Andrea O Rossetti, Sara Hocker, Teneille E Gofton, Nicholas S Abend, Emily J Gilmore, Cecil Hahn, Houman Khosravani, Felix Rosenow, Eugen Trinka
We convened an international group of experts to standardize definitions of New-Onset Refractory Status Epilepticus (NORSE), Febrile Infection-Related Epilepsy Syndrome (FIRES), and related conditions. This was done to enable improved communication for investigators, physicians, families, patients, and other caregivers. Consensus definitions were achieved via email messages, phone calls, an in-person consensus conference, and collaborative manuscript preparation. Panel members were from 8 countries and included adult and pediatric experts in epilepsy, electroencephalography (EEG), and neurocritical care...
April 2018: Epilepsia
Martin Holtkamp
Patients with prolonged seizures that do not respond to intravenous benzodiazepines and a second-line anticonvulsant suffer from refractory status epilepticus and those with seizures that do not respond to continuous intravenous anesthetic anticonvulsants suffer from super-refractory status epilepticus. Both conditions are associated with significant morbidity and mortality. A strict pharmacological treatment regimen is urgently required, but the level of evidence for the available drugs is very low. Refractory complex focal status epilepticus generally does not require anesthetics, but all intravenous non-anesthetizing anticonvulsants may be used...
March 2018: Drugs
Y Uchida, K Terada, Y Madokoro, T Fujioka, M Mizuno, T Toyoda, D Kato, N Matsukawa
BACKGROUND: Cross-sensitivity of rash has been reported between various antiepileptic drugs (AEDs). However, few studies have determined the frequency and management of cross-sensitivity in patients with super-refractory status epilepticus (SRSE). AIMS OF THE STUDY: To examine the optimal AED for treating SRSE with cross-sensitivity. METHODS: We performed a retrospective review of adult patients with SRSE treated at Nagoya City University Hospital, in which we investigated the frequency of cross-sensitivity among patients with SRSE and their clinical and medical profiles...
April 2018: Acta Neurologica Scandinavica
Usha K Misra, Jayantee Kalita, Deepanshu Dubey
Background: Super refractory status epilepticus (SRSE) is an important and recently recognized neurological emergency. Purpose: In view of paucity of studies on SRSE, we report the frequency, etiology and outcome of SRSE. Methods: In a hospital-based observational study during 2013 to 2016, consecutive patients with SRSE [persistence of status epilepticus (SE) for 24 h or more, or recurrence of SE on weaning of intravenous anesthetic] were included...
2017: Frontiers in Neurology
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