Determinants of mortality and long-term outcome in children with refractory and super refractory status epilepticus

Introduction. Refractory status epilepticus (RSE) has significant morbidity and mortality, and its management requires an accurate diagnosis and aggressive treatment. Objectives. To describe the experience of management of RSE in a neurological intensive care unit (NeuroICU) and determine predictors of short-term clinical outcome. Methods. We reviewed cases of RSE from September 2007 to December 2008. Management was titrated to findings on continuous video EEG (cVEEG). We collected patients’ demographics, RSE etiology, characteristics of seizures, cVEEG findings, treatments, and short-term outcome. Control of RSE was to achieve burst suppression pattern or electrographic cessation of ictal activity. Results. We included 80 patients; 63.8% were in coma, 25% had subclinical seizures, and 11.3% had focal activity. 51.3% were male and mean age was 45 years. Etiology was neurological lesion in 75.1%, uncontrolled epilepsy in 20%, and systemic derangements in 4.9%. 78.8% were treated with general anesthesia and concomitant anticonvulsant drugs. The control of RSE was 87.5% of patients. In-hospital mortality was 22.5%. The factors associated with unfavorable short-term outcome were coma and age over 60 years. Conclusions. RSE management guided by cVEEG is associated with a good seizure control. A multidisciplinary approach can help achieve a better short-term functional outcome in noncomatose patients.

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Long-term outcome of 32 children with encephalopathy with status epilepticus during sleep, or ESES syndrome

Epilepsia ◽

10.1111/j.1528-1167.2010.02578.x ◽

2010 ◽

Vol 51 (10) ◽

pp. 2023-2032 ◽

Cited By ~ 77

Author(s):

Elina Liukkonen ◽

Elisa Kantola-Sorsa ◽

Ritva Paetau ◽

Eija Gaily ◽

Maria Peltola ◽

...

Keyword(s):

Status Epilepticus ◽

Term Outcome ◽

Long Term Outcome

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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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SHORT AND LONG TERM OUTCOME OF STATUS EPILEPTICUS IN CHILDREN

Critical Care Medicine ◽

10.1097/00003246-200412001-00360 ◽

2004 ◽

Vol 32 (Supplement) ◽

pp. A100

Author(s):

Shaul Sofer ◽

Tali Turm ◽

Zamir Shorer ◽

Natalya Bilanko

Keyword(s):

Status Epilepticus ◽

Term Outcome ◽

Long Term Outcome ◽

Short And Long Term

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Status epilepticus severity influences the long-term outcome of neurogenesis in the adult dentate gyrus

Neurobiology of Disease ◽

10.1016/j.nbd.2003.11.010 ◽

2004 ◽

Vol 15 (2) ◽

pp. 196-205 ◽

Cited By ~ 90

Author(s):

Paul Mohapel ◽

Christine T Ekdahl ◽

Olle Lindvall

Keyword(s):

Status Epilepticus ◽

Dentate Gyrus ◽

Term Outcome ◽

Long Term Outcome

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Lack of Prominent Cognitive Regression in the Long-term Outcome of Patients Having Electrical Status Epilepticus During Sleep With Different Types of Epilepsy Syndromes

Clinical EEG and Neuroscience ◽

10.1177/1550059413514388 ◽

2014 ◽

Vol 46 (3) ◽

pp. 235-242 ◽

Cited By ~ 2

Author(s):

Nerses Bebek ◽

Candan Gürses ◽

Betül Baykan ◽

Aysen Gökyiğit

Keyword(s):

Status Epilepticus ◽

Term Outcome ◽

Long Term Outcome ◽

Different Types

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Long-term risk of chronic epilepsy in adults after post-anoxic coma and refractory status epilepticus: a retrospective cohort study

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2020-324767 ◽

2020 ◽

pp. jnnp-2020-324767

Author(s):

Lorenzo Tinti ◽

Simone Beretta ◽

Anna Coppo ◽

Elisa Bianchi ◽

Clara Zanchi ◽

...

Keyword(s):

Cohort Study ◽

Status Epilepticus ◽

Retrospective Cohort Study ◽

Retrospective Cohort ◽

Refractory Status Epilepticus ◽

Chronic Epilepsy ◽

Refractory Status ◽

Anoxic Coma

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Updates in Refractory Status Epilepticus

Critical Care Research and Practice ◽

10.1155/2018/9768949 ◽

2018 ◽

Vol 2018 ◽

pp. 1-19 ◽

Cited By ~ 4

Author(s):

Rohit Marawar ◽

Maysaa Basha ◽

Advait Mahulikar ◽

Aaron Desai ◽

Kushak Suchdev ◽

...

Keyword(s):

Status Epilepticus ◽

Treatment Options ◽

Neurological Status ◽

Refractory Status Epilepticus ◽

Focal Lesion ◽

Long Term Outcomes ◽

Refractory Status ◽

Autoimmune Etiology ◽

New Onset

Refractory status epilepticus is defined as persistent seizures despite appropriate use of two intravenous medications, one of which is a benzodiazepine. It can be seen in up to 40% of cases of status epilepticus with an acute symptomatic etiology as the most likely cause. New-onset refractory status epilepticus (NORSE) is a recently coined term for refractory status epilepticus where no apparent cause is found after initial testing. A large proportion of NORSE cases are eventually found to have an autoimmune etiology needing immunomodulatory treatment. Management of refractory status epilepticus involves treatment of an underlying etiology in addition to intravenous anesthetics and antiepileptic drugs. Alternative treatment options including diet therapies, electroconvulsive therapy, and surgical resection in case of a focal lesion should be considered. Short-term and long-term outcomes tend to be poor with significant morbidity and mortality with only one-third of patients reaching baseline neurological status.

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