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Super refractory status epilepticus

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
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...
October 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)...
September 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...
September 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
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