New-onset super-refractory status epilepticus

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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New-onset super refractory status epilepticus: A case-series

Seizure ◽

10.1016/j.seizure.2019.10.005 ◽

2020 ◽

Vol 75 ◽

pp. 174-184 ◽

Cited By ~ 3

Author(s):

Sidra Aurangzeb ◽

Lara Prisco ◽

Jane Adcock ◽

Mahiri Speirs ◽

Simon Raby ◽

...

Keyword(s):

Status Epilepticus ◽

Case Series ◽

Refractory Status Epilepticus ◽

Refractory Status ◽

New Onset

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Infantile hemiconvulsion-hemiplegia epilepsy syndrome associated with GRIN2A gene mutation

Srpski arhiv za celokupno lekarstvo ◽

10.2298/sarh201125049k ◽

2021 ◽

pp. 49-49

Author(s):

Ruzica Kravljanac ◽

Slobodan Gazikalovic ◽

Biljana Vucetic-Tadic

Keyword(s):

Status Epilepticus ◽

Right Hemisphere ◽

Refractory Status Epilepticus ◽

Epilepsy Syndrome ◽

Refractory Status ◽

Specific Syndrome ◽

First Time ◽

Infectious Encephalitis ◽

New Onset

Introduction. Infantile hemiconvulsion-hemiplegia and epilepsy (IHHE) syndrome is defined as a specific syndrome in a patient < 2 years of age, presenting as a new onset refractory status epilepticus with unilateral motor seizures and acute imaging abnormalities, fever, hemiparesis >24 hours, and excluding infectious encephalitis. Case outline. We present results of follow-up in 11 year-old girl with IHHE, associated with GRIN2A mutation. The girl had normal development until the first febrile hemiconvulsive status epilepticus at the age of seven months. Neuroimaging initially showed right hemisphere edema, followed by progressive right side hemiatrophy. The patient has resistant epilepsy, left side hemiparesis, and good language and cognitive development. Conclusion. Despite IHHE described many years ago, some syndrome's features, including etiology have remained unexplained. The association of IHHE and GRIN2A mutation is described in current manuscript for the first time in scientific literature.

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In Small Case Series, Tocilizumab Shows Promise for New-Onset Refractory Status Epilepticus

Neurology Today ◽

10.1097/01.nt.0000552941.54468.3b ◽

2019 ◽

Vol 19 (1) ◽

pp. 9-10

Author(s):

Gina Shaw

Keyword(s):

Status Epilepticus ◽

Case Series ◽

Refractory Status Epilepticus ◽

Small Case Series ◽

Refractory Status ◽

New Onset

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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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Intravenous immunoglobulin response in new-onset refractory status epilepticus (NORSE) COVID-19 adult patients

Journal of Neurology ◽

10.1007/s00415-021-10468-y ◽

2021 ◽

Cited By ~ 1

Author(s):

Paolo Manganotti ◽

Giovanni Furlanis ◽

Miloš Ajčević ◽

Cristina Moras ◽

Lucia Bonzi ◽

...

Keyword(s):

Status Epilepticus ◽

Intravenous Immunoglobulin ◽

Case Series ◽

Autoimmune Encephalitis ◽

Refractory Status Epilepticus ◽

Adult Patients ◽

University Hospital ◽

Paroxysmal Activity ◽

Refractory Status ◽

New Onset

AbstractNeurological manifestations may be common in COVID-19 patients. They may include several syndromes, such as a suggested autoimmune abnormal response, which may result in encephalitis and new-onset refractory status epilepticus (NORSE). Quickly recognizing such cases and starting the most appropriate therapy is mandatory due to the related rapid worsening and bad outcomes. This case series describes two adult patients admitted to the university hospital and positive to novel coronavirus 2019 (SARS-CoV-2) infection who developed drug-resistant status epilepticus. Both patients underwent early electroencephalography (EEG) assessment, which showed a pathological EEG pattern characterized by general slowing, rhythmic activity and continuous epileptic paroxysmal activity. A suspected autoimmune etiology, potentially triggered by SARS-CoV-2 infection, encouraged a rapid work-up for a possible autoimmune encephalitis diagnosis. Therapeutic approach included the administration of 0.4 g/kg intravenous immunoglobulin, which resulted in a complete resolution of seizures after 5 and after 10 days, respectively, without adverse effects and followed by a normalization of the EEG patterns.

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How to Help Your Patients Enroll in the New-Onset Refractory Status Epilepticus (NORSE) and Febrile Infection-Related Epilepsy Syndrome (FIRES) Family Registry, and Other Rare Epilepsy Registries

Epilepsy Currents ◽

10.1177/1535759721998329 ◽

2021 ◽

pp. 153575972199832

Author(s):

Karnig Kazazian ◽

Marissa Kellogg ◽

Nora Wong ◽

Krista Eschbach ◽

Raquel Farias Moeller ◽

...

Keyword(s):

Status Epilepticus ◽

Refractory Status Epilepticus ◽

Epilepsy Syndrome ◽

Neurologic Disorder ◽

Mortality And Morbidity ◽

Refractory Status ◽

Rigorous Research ◽

Active Epilepsy ◽

New Onset

New-onset refractory status epilepticus (NORSE) is a rare clinical presentation of refractory status epilepticus (RSE) that occurs in people without active epilepsy or preexisting neurologic disorder. Febrile infection-related epilepsy syndrome (FIRES) is a subcategory of NORSE. New-onset refractory status epilepticus/FIRES are becoming increasingly recognized; however, information pertaining to disease course, clinical outcomes, and survivorship remains limited, and mortality and morbidity are variable, but often high. The objective of the NORSE/FIRES Family Registry is to (1) provide an easily accessible and internationally available multilingual registry into which survivors or NORSE/FIRES surrogates or family members of people affected by NORSE/FIRES or their physicians can enter data in a systematic and rigorous research study from anywhere in the world where internet is available; and (2) to examine past medical history, outcomes, and quality of life for people affected by NORSE/FIRES.

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