Super-refractory status epilepticus (SRSE): A case series of 22 pediatric patients

Super-refractory status epilepticus (SRSE) represents a neurological emergency that is characterized by a lack of response to the third line of antiepileptic treatment, including intravenous general anesthetics. It is a medical challenge with high morbidity and mortality. Electroconvulsive therapy (ECT) has been recommended as a nonpharmacologic option of treatment after other alternatives are unsuccessful. Its effect on the cessation of SRSE has been minimally investigated. The objective of this article is to analyze the effect of ECT on SRSE. For this purpose, a multidisciplinary team created a protocol based on clinical guidelines similar to those described previously by Ray et al. (2017). ECT was applied to six patients with SRSE after the failure of antiepileptic treatment and pharmacologic coma.The objective of each ECT session was to elicit a motor seizure for at least 20 s. SRSE was resolved in all patients after several days of treatment, including ECT as a therapy, without relevant adverse effects. Thus, ECT is an effective and feasible option in the treatment of SRSE, and its place in the algorithm in treatment should be studied due to the uncommon adverse effects and the noninvasive character of the therapy.

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Intravenous Immunoglobulins for Refractory Status Epilepticus: A Scoping Systematic Review of the Pediatric Literature

Journal of Pediatric Neurology ◽

10.1055/s-0037-1603594 ◽

2017 ◽

Vol 15 (06) ◽

pp. 305-315

Author(s):

M. Matuszczak ◽

J. Teitelbaum ◽

C. Kazina ◽

L. Gillman ◽

F. Zeiler

Keyword(s):

Systematic Review ◽

Status Epilepticus ◽

Pediatric Patients ◽

Ivig Therapy ◽

Intravenous Immunoglobulins ◽

Refractory Status Epilepticus ◽

Neurological Deficits ◽

Cochrane Library ◽

Refractory Status ◽

Grade Methodology

AbstractOur goal was to perform a scoping systematic review of the literature on the use of intravenous immunoglobulins (IVIGs) for refractory status epilepticus (RSE) in pediatric patients. Articles from MEDLINE, BIOSIS, EMBASE, Global Health, Healthstar, Scopus, Cochrane Library, the International Clinical Trials Registry Platform, ClinicalTrials.gov (inception to June 2016), reference lists of relevant articles, and gray literature were searched. The strength of evidence was adjudicated using both the Oxford and Grading of Recommendation Assessment Development and Education (GRADE) methodology by two independent reviewers. We identified 21 original articles. Eighty-seven pediatric patients were described as having received IVIG therapy for RSE. The mean age was 7.8 years (range: 2–17.5 years). Seizure response occurred in 14 of the 87 patients (16.1%), with 3 (3.4%) and 11 (12.6%) displaying partial and complete responses, respectively. Seventy-three of the 87 patients (83.9%) failed to display any seizure response to IVIG therapy. No complications related to IVIG therapy were recorded. The majority of patients had moderate to severe neurological deficits upon follow-up. Oxford level 4, GRADE D evidence exists to suggest little to no impact on seizure control in pediatric autoimmune RSE. Routine use of IVIG for pediatric RSE cannot be recommended at this time and should be considered experimental.

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The Treatment and Prognosis of Refractory and Super-Refractory Status Epilepticus

Neurology International Open ◽

10.1055/s-0043-110165 ◽

2017 ◽

Vol 01 (03) ◽

pp. E204-E210

Author(s):

Stephanie Gollwitzer ◽

Hajo Hamer

Keyword(s):

Status Epilepticus ◽

Electroconvulsive Therapy ◽

Case Reports ◽

Case Series ◽

Refractory Status Epilepticus ◽

Evidence Based ◽

New Approach ◽

Refractory Status ◽

First Choice ◽

Evidence Based Guidelines

AbstractRefractory status epilepticus (RSE) is defined as status epilepticus (SE) persisting over 60 min and resistant to treatment with benzodiazepines and non-sedating antiepileptic drugs. The term super-refractory status epilepticus (SRSE) refers to a refractory episode continuing under general anesthesia for more than 24 h. RSE is treated with a combination of non-sedating AED and i. v. anesthetics; first choice drugs are midazolam, propofol and thiopental. The management of super-refractory status epilepticus (SRSE) is challenging as clear evidence-based guidelines are lacking. Recommendations are mainly based on case reports and small case series. Therapeutic options include ketamine, inhalational anesthetics, steroids and immunoglobulins. Ketogenic diet, electroconvulsive therapy and epilepsy surgery are also considered as potentially effective. A promising new approach is the neurosteroid allopregnanolone. Mortality of RSE and SRSE is largely influenced by the etiology and is markedly higher as compared to non-refractory status epilepticus. It was reported to be about 30% and 50%, respectively.

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Super-Refractory Status Epilepticus Treated with High Dose Perampanel: Case Series and Review of the Literature

Case Reports in Critical Care ◽

10.1155/2019/3218231 ◽

2019 ◽

Vol 2019 ◽

pp. 1-7

Author(s):

Christopher R. Newey ◽

Naresh Mullaguri ◽

Stephen Hantus ◽

Vineet Punia ◽

Pravin George

Keyword(s):

Cardiac Arrest ◽

Status Epilepticus ◽

Case Series ◽

Refractory Status Epilepticus ◽

Critically Ill Patient ◽

Unknown Etiology ◽

High Dose ◽

The Novel ◽

Refractory Status ◽

Symptomatic Seizures

Introduction. Acute symptomatic seizures are frequent in the critically ill patient and can be difficult to treat. The novel anticonvulsant perampanel may be effective in the treatment of status epilepticus considering its mechanism of action of being an AMPA antagonist. We present four cases of super refractory status epilepticus treated with high dose perampanel. Method. Case report. Cases. Four patients were treated with perampanel for their refractory status epilepticus. One patient had new onset refractory status epilepticus of unknown etiology. Three other patients had status epilepticus as a result of their cardiac arrest. Two of the cardiac arrest patients had myoclonus. In all patients, the additional of perampanel resulted in a reduction of seizure burden without affecting hemodynamics or hepatic or renal function. Conclusion. Perampanel may be effective in the treatment of super-refractory status epilepticus of varying etiologies. A larger, prospective study is needed to further assess this therapy.

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New-onset super-refractory status epilepticus

Neurology ◽

10.1212/wnl.0000000000010787 ◽

2020 ◽

Vol 95 (16) ◽

pp. e2280-e2285 ◽

Cited By ~ 1

Author(s):

Elizabeth Matthews ◽

Ayham Alkhachroum ◽

Nina Massad ◽

Riva Letchinger ◽

Kevin Doyle ◽

...

Keyword(s):

Status Epilepticus ◽

Medical Center ◽

Case Series ◽

Refractory Status Epilepticus ◽

Hospital Length Of Stay ◽

Post Discharge ◽

Refractory Status ◽

New Onset

ObjectiveTo better understand the heterogeneous population of patients with new-onset refractory status epilepticus (NORSE), we studied the most severe cases in patients who presented with new-onset super-refractory status epilepticus (NOSRSE).MethodsWe report a retrospective case series of 26 adults admitted to the Columbia University Irving Medical Center neurologic intensive care unit (NICU) from February 2009 to February 2016 with NOSRSE. We evaluated demographics, diagnostic studies, and treatment course. Outcomes were modified Rankin Scale score (mRS) at hospital discharge and most recent follow-up visit (minimum of 2 months post discharge), NICU and hospital length of stay, and long-term antiepileptic drug use.ResultsOf the 252 patients with refractory status epilepticus, 27/252 had NORSE and 26/27 of those had NOSRSE. Age was bimodally distributed with peaks at 27 and 63 years. The majority (96%) had an infectious or psychiatric prodrome. Etiology was cryptogenic in 73%, autoimmune in 19%, and infectious in 8%. Seven patients (27%) underwent brain biopsy, autopsy, or both; 3 (12%) were diagnostic (herpes simplex encephalitis, candida encephalitis, and acute demyelinating encephalomyelitis). On discharge, 6 patients (23%) had good or fair outcome (mRS 0–3). Of the patients with long-term follow-up data (median 9 months, interquartile range 2–22 months), 12 patients (71%) had mRS 0–3.ConclusionAmong our cohort, nearly all patients with NORSE had NOSRSE. The majority were cryptogenic with few antibody-positive cases identified. Neuropathology was diagnostic in 12% of cases. Although only 23% of patients had good or fair outcome on discharge, 71% met these criteria at follow-up.

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