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'The great imitator': Neurosyphilis and new-onset refractory status epilepticus (NORSE) syndrome.

ABSTRACT: New-onset refractory status epilepticus (NORSE) is a syndrome of new-onset drug resistant status epilepticus that often has a catastrophic outcome. A 30-year-old man of Somali origin presented with refractory status to a district general hospital. A clinical diagnosis of NORSE syndrome was made, and he was transferred to the regional epilepsy center for immunomodulatory treatment and consideration for cyclophosphamide treatment. After transfer to the regional epilepsy center, his repeat cerebrospinal fluid tested strongly positive for syphilis, indicating a diagnosis of neurosyphilis, and the patient was treated with high-dose intravenous (IV) benzylpenicillin. His status epilepticus abated 24 h later. New-onset refractory status epilepticus syndrome is a diagnosis of exclusion. Before instigation of potentially harmful neuromodulatory therapies, treatable causes such as neurosyphilis should be considered. We advocate the early transfer of refractory status patients to a specialist epilepsy center for both seizure management and cause determination.

SUBMITTER: Kumari S

PROVIDER: S-EPMC4388970 | biostudies-literature |

REPOSITORIES: biostudies-literature

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New-onset refractory status epilepticus (NORSE) in post SARS-CoV-2 autoimmune encephalitis: a case report.

Project description:The 2019 new coronavirus (SARS-CoV-2) is a novel respiratory virus which has increasingly spread all over the world. Although the predominant clinical presentation is represented by respiratory symptoms, neurological manifestation of SARS-CoV-2 is being increasingly recognized. In the present report, we present a case of post SARS-CoV-2 autoimmune encephalitis associated with a new-onset refractory status epilepticus (NORSE).

| S-EPMC7608104 | biostudies-literature

International consensus recommendations for management of New Onset Refractory Status Epilepticus (NORSE) including Febrile Infection-Related Epilepsy Syndrome (FIRES): Summary and Clinical Tools.

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| S-EPMC9826478 | biostudies-literature

International consensus recommendations for management of New Onset Refractory Status Epilepticus (NORSE) incl. Febrile Infection-Related Epilepsy Syndrome (FIRES): Statements and Supporting Evidence.

Project description:ObjectiveTo develop consensus-based recommendations for the management of adult and paediatric patients with NORSE/FIRES based on best evidence and experience.MethodsThe Delphi methodology was followed. A facilitator group of 9 experts was established, who defined the scope, users and suggestions for recommendations. Following a review of the current literature, recommendation statements concerning diagnosis, treatment and research directions were generated which were then voted on a scale of 1 (strongly disagree) to 9 (strongly agree) by a panel of 48 experts in the field. Consensus that a statement was appropriate was reached if the median score was greater or equal to 7, and inappropriate if the median score was less than or equal to 3. The analysis of evidence was mapped to the results of each statement included in the Delphi survey.ResultsOverall, 85 recommendation statements achieved consensus. The recommendations are divided into five sections: 1) disease characteristics, 2) diagnostic testing and sampling, 3) acute treatment, 4) treatment in the post-acute phase, and 5) research, registries and future directions in NORSE/FIRES. The detailed results and discussion of all 85 statements are outlined herein. A corresponding summary of findings and practical flowsheets are presented in a companion article.SignificanceThis detailed analysis offers insight into the supporting evidence and the current gaps in the literature that are associated with expert consensus statements related to NORSE/FIRES. The recommendations generated by this consensus can be used as a guide for the diagnosis, evaluation, and management of patients with NORSE/FIRES, and for planning of future research.

| S-EPMC9828002 | biostudies-literature

New-onset refractory status epilepticus: Etiology, clinical features, and outcome.

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| S-EPMC4642147 | biostudies-literature

Perspective: Vagal nerve stimulation in the treatment of new-onset refractory status epilepticus.

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| S-EPMC10157031 | biostudies-literature

Unraveling the enigma of new-onset refractory status epilepticus: a systematic review of aetiologies.

Project description:Background and purposeNew-onset refractory status epilepticus (NORSE) is a clinical presentation, neither a specific diagnosis nor a clinical entity. It refers to a patient without active epilepsy or other pre-existing relevant neurological disorder, with a NORSE without a clear acute or active structural, toxic or metabolic cause. This study reviews the currently available evidence about the aetiology of patients presenting with NORSE and NORSE-related conditions.MethodsA systematic search was carried out for clinical trials, observational studies, case series and case reports including patients who presented with NORSE, febrile-infection-related epilepsy syndrome or the infantile hemiconvulsion-hemiplegia and epilepsy syndrome.ResultsFour hundred and fifty records were initially identified, of which 197 were included in the review. The selected studies were retrospective case-control (n = 11), case series (n = 83) and case reports (n = 103) and overall described 1334 patients both of paediatric and adult age. Aetiology remains unexplained in about half of the cases, representing the so-called 'cryptogenic NORSE'. Amongst adult patients without cryptogenic NORSE, the most often identified cause is autoimmune encephalitis, either non-paraneoplastic or paraneoplastic. Infections are the prevalent aetiology of paediatric non-cryptogenic NORSE. Genetic and congenital disorders can have a causative role in NORSE, and toxic, vascular and degenerative conditions have also been described.ConclusionsFar from being a unitary condition, NORSE is a heterogeneous and clinically challenging presentation. The development and dissemination of protocols and guidelines to standardize diagnostic work-up and guide therapeutic approaches should be implemented. Global cooperation and multicentre research represent priorities to improve the understanding of NORSE.

| S-EPMC9298123 | biostudies-literature

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Project description: Not available

| S-EPMC7362825 | biostudies-literature

New-Onset Refractory Status Epilepticus with Claustrum Damage: Definition of the Clinical and Neuroimaging Features.

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Treatment of Refractory and Super-refractory Status Epilepticus.

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