Five cases of new onset refractory status epilepticus (NORSE) syndrome: Outcomes with early immunotherapy

Gall, Claire; Jumma, Odai; Mohanraj, Rajiv

doi:10.1016/j.seizure.2012.12.016

Cited by 83 publications

(107 citation statements)

References 21 publications

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“…Early immunotherapy with intravenous methylprednisolone (IvMP), intravenous immunoglobulin (IvIg) or both, was recently reported to be efficient in three of five adults suffering from new-onset refractory SE who received these medications, whereas the two other patients of the series who did not receive immunomodulatory treatments had a more severe outcome. The results of previous case descriptions with similar approaches described in that article were less convincing, however [85]. Corticosteroids are used as first-line immunotherapy and have their place in the treatment of SE even in the absence of an autoimmune etiology, since SE may be associated with an inflammatory response and with a disruption of the BBB [105].…”

Section: Autoimmunity and Inflammation In Status Epilepticusmentioning

confidence: 88%

Autoimmunity and inflammation in status epilepticus: from concepts to therapies

Holzer

Seeck

Korff³

2014

Expert Review of Neurotherapeutics

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The understanding of immunological mechanisms underlying some forms of epilepsy and encephalitis has rapidly increased for the last 10 years leading to the concept of status epilepticus of autoimmune origin. Actual treatment recommendations regarding autoimmune status epilepticus are based on retrospective case studies, pathophysiological considerations and experts' opinion. In addition, there are no clear indicators to predict outcome. In situations where autoimmune mechanisms are suspected in patients with status epilepticus, there is evidence that earlier treatment is related to better outcome. Increased awareness is mandatory to decrease the number of patients with major neurological problems or fatal outcome, which is overall about 50%. We here summarize findings of all pediatric and adult patients reported to date, and review the current state of knowledge in the field of immune therapeutic approaches of status epilepticus.

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Section: Autoimmunity and Inflammation In Status Epilepticusmentioning

confidence: 88%

Autoimmunity and inflammation in status epilepticus: from concepts to therapies

Holzer

Seeck

Korff³

2014

Expert Review of Neurotherapeutics

View full text Add to dashboard Cite

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“…The studies included are highlighted in Table 1. A retrospective analysis of five patients with NORSE by Gall et al showed that three out of five patients who received immunotherapy (steroids, IVIg, and/or azathioprine) had seizures subsequently controlled on AEDs, with only one patient developing mild cognitive decline [9]. The fourth patient died (no IT administered), and the fifth patient's outcome was unknown.…”

Section: Discussionmentioning

confidence: 99%

New-onset refractory status epilepticus (NORSE) — The potential role for immunotherapy

Khawaja

DeWolfe

Miller

et al. 2015

Epilepsy & Behavior

109

122

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“…This has prompted the eponym NORSE, for New Onset Refractory Status Epilepticus (Wilder-Smith et al, 2005). We found four published series of NORSE in adults (Costello et al, 2009;Gall et al, 2013;Van Lierde et al, 2003;Wilder-Smith et al, 2005) (Table 2). Altogether, they included 24 young, previously healthy patients with RSE lasting Two remained impaired and dependent Two remained impaired but were independent One had a "good outcome" Three survivors had refractory epilepsy; one was seizure-free on 2 ASDs; one was seizurefree without any ASD (Continued) from 6 to 191 days.…”

Section: Cryptogenic Refractory Status Epilepticus or New Onset Refrmentioning

confidence: 90%

Is Favorable Outcome Possible After Prolonged Refractory Status Epilepticus?

Alvarez

Drislane

2016

Journal of Clinical Neurophysiology

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When status epilepticus (SE) remains refractory to appropriate therapy, it is associated with high mortality and with substantial morbidity in survivors. Many outcome predictors such as age, seizure type, level of consciousness before treatment, and mostly, etiology, are well-established. A longer duration of SE is often associated with worse outcome, but duration may lose its prognostic value after several hours. Several terms and definitions have been used to describe prolonged, refractory SE, including "malignant SE," "prolonged" SE, and more recently, "super refractory" SE, defined as "SE that has continued or recurred despite 24 hours of general anesthesia (or coma-inducing anticonvulsants)." There are few data available regarding the outcome of prolonged refractory SE, and even fewer for SE remaining refractory to anesthetic drugs. This article reviews reports of outcome after prolonged, refractory, and "super refractory" SE. Most information detailing the clinical outcome of patients surviving these severe illnesses, in which seizures can persist for days or weeks (and especially those concerning "super-refractory" SE) come from case reports and retrospective cohort studies. In many series, prolonged, refractory SE has a mortality of 30% to 50%, and several studies indicate that most survivors have a substantial decline in functional status. Nevertheless, several reports demonstrate that good functional outcome is possible even after several days of SE and coma induction. Treatment of refractory SE should not be withdrawn from younger patients without structural brain damage at presentation solely because of the duration of SE.

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Five cases of new onset refractory status epilepticus (NORSE) syndrome: Outcomes with early immunotherapy

Abstract: In our experience, early immunotherapy has been associated with good outcomes in NORSE. Multicentre collaboration is required to establish the diagnostic criteria and appropriate management of patients presenting with NORSE.

Cited by 83 publications

References 21 publications

Autoimmunity and inflammation in status epilepticus: from concepts to therapies

Autoimmunity and inflammation in status epilepticus: from concepts to therapies

New-onset refractory status epilepticus (NORSE) — The potential role for immunotherapy

Is Favorable Outcome Possible After Prolonged Refractory Status Epilepticus?

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