Treatment of refractory and superrefractory status. Management of refractory status epilepticus involves treatment of an. Refractory status epilepticus occurs in 1070% of adults. In this episode of hallway conversations, editorinchief dr. Newonset refractory status epilepticus norse is defined as a condition, not a specific diagnosis, with new onset of refractory status epilepticus without a clear acute or active structural, toxic or metabolic cause in a patient without active epilepsy. Management of patients with status epilepticus generally requires several.
A definition and classification of status epilepticus report of the ilae task force on classification of status epilepticus. Febrile infectionrelated epilepsy syndrome fires is a rare but devastating epileptic encephalopathy typically affecting schoolaged children, characterized by a preceding febrile illness, followed by multifocal seizures and often refractory status epilepticus rse 1. Status epilepticus may occur in those with a history of epilepsy as well as those with an underlying problem of the brain. Ketogenic diet in 3 cases of childhood refractory status epilepticus. Transitioning treatment from status epilepticus to super refractory status epilepticus david roy, pharmd pgy1 pharmacy resident. The term super refractory status epilepticus was introduced during the londoninnsbruck colloquium on status epilepticus in 2011 and refers to status epilepticus that continues or recurs 24 h or. Because of the small numbers, there are no definitive studies regarding. Newonset refractory status epilepticus norse is a recently coined term for refractory status epilepticus where. Superrefractory status epilepticus explained youtube.
Refractory status epilepticus is a potentially lifethreatening medical emergency. These underlying brain problems may include trauma, infections, or strokes among others. Evolution of cerebral atrophy in a patient with super. There are a variety of treatments used, almost entirely based on open observational studies or case reports. Jan 14, 2015 frequency of refractory status epilepticus in status epilepticus patients 3144% no community based incidence studies in india but is expected to be high because of high prevalence of epilepsy, cns infections and treatment gap. Immune therapies were used less frequently in cryptogenic cases, despite a comparable prevalence of inflammatory csf changes. Superrefractory status epilepticusstatus epilepticus that continues or recurs 24 hours or more after the onset of anesthetic therapyis. Outcome at discharge is poor but improves during followup.
Status epilepticus is a neurologic emergency and can progress to refractory status epilepticus and super refractory status epilepticus. Generalized convulsive status epilepticus emcrit project. Gaps and opportunities in refractory status epilepticus. How refractory is super refractory status epilepticus a personal iew. Superrefractory status epilepticus is defined as status epilepticus that continues or recurs 24h. This is a pdf file of an unedited manuscript that has been. The term superrefractory status epilepticus srse refers to a re fractory episode continuing under general anesthesia for more than 24 h. However, limited evidence supports to a specific treatment that helps in curing. Several recent guidelines and consensus statements highlight the critical role of continuous eeg ceeg monitoring during prolonged treatment. Status epilepticus is a lifethreatening medical emergency, particularly if treatment is delayed.
Treatment of refractory and superrefractory status epilepticus. Treatment of refractory status epilepticus stephan schuele, md, mph northwestern university chicago, il definition refractory status epilepticus rse is defined as seizure activity which persists for over 2 hours and after failing two first line treatments, usually a benzodiazepine and a conventional intravenous antiepileptic medication. The treatment and prognosis of refractory and super. The different electroclinical types of status epilepticus share eeg characteristics. The norse newonset refractory status epilepticus syndrome. Management of refractory status epilepticus in adults.
The cerebral atrophy was noticeable at 31 days after admission at our facility which emphasizes the urgency of definitive treatment in patients who present with super refractory status epilepticus. Electroencephalography is the gold standard for diagnosis. How refractory is superrefractory status epilepticus a. Tracy glauser, from the university of cincinnati, about new status epilepticus guidelines. Refractory status epilepticus is defined as status epilepticus that persists despite. Treatment of rse has not been studied prospectively, and guidelines give a variety of options.
Newonset refractory status epilepticus norse and febrile. It is likewise necessary to attend to the refractory status epilepticus promptly to prevent mortality. It is a serious condition that requires prompt medical attention. Superrefractory status epilepticus and febrile infection. The american epilepsy society guidelines published in. There is a lack of consensus upon its semantic definition of whether it is status epilepticus that continues despite treatment with benzodiazepine and one antiepileptic medication aed, i. New status epilepticus guidelines epilepsy foundation. References status epilepticus care guideline agarwal p, et al. The treatment of superrefractory status epilepticus. Objective to investigate neurologic outcome of patients with cardiac arrest with refractory status epilepticus rse treated with a standardized aggressive protocol with antiepileptic drugs and anesthetics compared to patients with other eeg patterns. Many factors often combine to lower the patients seizure threshold. In status epilepticus seizures appear in close succession or dont stop.
Some authorities have also defined rse on the basis of the duration of seizure for 1 or 2 hours. Super refractory status epilepticus is defined as status epilepticus that continues or recurs 24 h or more after the onset of anaesthetic therapy, including those cases where status epilepticus recurs on the reduction or withdrawal of anaesthesia. Status epilepticus international league against epilepsy. Refractory status epilepticus is defined as persistent seizures despite appropriate use of two intravenous medications, one of which is a benzodiazepine. Refractory and superrefractory status epilepticus indian pediatrics. Treatment of refractory and superrefractory status epilepticus ncbi. Predictors of outcome in refractory status epilepticus. It is an uncommon but important clinical problem with high mortality and morbidity rates. The term superrefractory status epilepticus was introduced during the londoninnsbruck colloquium on status epilepticus in 2011 and refers to status epilepticus that continues or. Randomized study of intravenous valproate and phenytoin in status epilepticus. The treatment of superrefractory status epilepticus neurology. Additionally gene expression within few minutes of seizures and lack of synchrony.
Management of superrefractory status epilepticus with. The concept of super refractory status and newer etiologies and therapeutic options are also discussed. This case highlights the development and progression of generalized cerebral atrophy in super refractory status epilepticus. Md of massachusetts general hospital and harvard medical school explains super refractory status epilepticus. Methods in the prospective cohort study, 166 consecutive patients with cardiac arrest in coma were stratified according to 4 independent eeg. With the exception of anaesthetic coma, which is not sustainable, and the ketogenic diet in some patients 2, 3. The article summarises the causes, effects, pathophysiology and treatment protocols for refractory status epilepticus. Jan 28, 2015 super refractory status epilepticus srse is defined as status epilepticus that continues 24 h or more after the onset of anesthesia, and includes those cases in which epilepsy is recurrent upon treatment reduction. Cole epilepsy service, department of neurology, acc 835, massachusetts general hospital, harvard medical school, boston, ma 02114, usa.
Nov 08, 2017 refractory status epilepticus falls under the status epilepticus category where the patient continues to show the epilepticus even while continuing the treatment using benzodiazepines and antiepileptic medicines. 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. Cryptogenic new onset refractory status epilepticus norse in adultsinfectious or not. Apr 21, 2016 super refractory status epilepticus explained rare disease report. Autoimmune encephalitis is the most commonly identified cause of newonset refractory status epilepticus, but half remain cryptogenic. Nicholas hirsch neuromedical intensive care unit the national hospital for neurology and neurosurgery queen square london state of the art meeting 2015 2. Pdf refractory and superrefractory status epilepticusan.
Cryptogenic new onset refractory status epilepticus norse. Refractory status epilepticus rse has been defined variably in the literature as seizures that do not respond to 2 14 or 3 58 different antiepileptic medications, usually intravenous benzodiazepines as a firstline agent and intravenous phenytoin, phenobarbital, or valproic acid as a secondline agent. Neurologic outcome of postanoxic refractory status. Super refractory status epilepticus srse is defined as status epilepticus that continues 24 h or more after the onset of anesthesia, and includes those cases in which epilepsy is recurrent upon treatment reduction. Treatment of rse has not been studied in a prospective trial, and. The exact definition is still unclear, with different studies defining refractory status epilepticus with varying durations no time criteria, 30 minutes, 1 hour, or 2 hours and a lack of response to numbers two or three and types of medications. Refractory status epilepticus is defined as persistent seizures despite. Super refractory status epilepticus how long should we persevere. Super refractory status epilepticus status epilepticus that continues or recurs 24 hours or more after the onset of anesthetic therapyis a medical emergency. Refractory status epilepticus rse can be defined as status epilepticus that. Status epilepticus, refractory status epilepticus, and. Newonset refractory status epilepticus johns hopkins.
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