New onset refractory status epilepticus: State of the art

Périn, Bertille; Szurhaj, William

doi:10.1016/j.neurol.2021.12.005

Cited by 5 publications

(8 citation statements)

References 68 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Several articles have proclaimed older age groups beyond 60 to be at high risk of NORSE. [10][11][12] Unlike the literature, NCSE has been a common finding seen amongst 58.3% of patients, whereas in NORSE the most common type of seizure seen is FMS, which was present in just 1 (8.3%) patient from our cohort. NCSE is the absence of any prominent motor signs sparing the minor abnormal repetitive movements.…”

Section: Discussioncontrasting

confidence: 67%

New-Onset Refractory Status Epilepticus Secondary to COVID-19 Infection in Adults: A Systematic Review

Arif¹,

Chavarria²,

Qamar³

et al. 2022

NDT

View full text Add to dashboard Cite

Background New-onset refractory status epilepticus (NORSE) has been reported in the scientific literature as a phenomenon associated with the COVID-19 infection. Given the resurgence of the newer variants of COVID-19 added with its multi-system manifestations, this project was conducted to study the clinical picture of NORSE secondary to COVID-19 infection. Methods Three electronic databases were searched using an extensive search strategy from November 2019 to December 2021. Patients reporting NORSE secondary to COVID-19 were included in this review. The status epilepticus severity score (STESS) was calculated by the study authors for individual patients. Statistical analysis was performed using SPSS version 26 with a p -value <0.05 as statistically significant. Results After screening, 12 patients were included in this study with a mean age of 61.6 ± 19.0-year olds. The most common type of status epilepticus reported in our study population was non-convulsive status epilepticus (NCSE) (7 out of 12 patients, 58.3%). The linear regression model revealed that STESS scores were significantly influenced by patients’ age ( p = 0.004) and intra-hospital occurrence (IHO) of status epilepticus ( p = 0.026). Overall, 8 patients (66.7%) were discharged without complications. Conclusion Given the observed association of STESS with the aging population and IHO of status epilepticus, special attention is due to the caretakers of this population, while further studies are needed to further build upon this review.

show abstract

Section: Discussioncontrasting

confidence: 67%

New-Onset Refractory Status Epilepticus Secondary to COVID-19 Infection in Adults: A Systematic Review

Arif¹,

Chavarria²,

Qamar³

et al. 2022

NDT

View full text Add to dashboard Cite

show abstract

“…8 But a substantial number of cases remain cryptogenic. 9 Outcomes in patients with FIRES and NORSE are generally poor, with greater than 10% mortality 10 and longterm neurological sequelae in survivors ranging from cognitive delay and impairment to severe and irreversible encephalopathic brain damage. 5 Permanent drug-resistant epilepsy is a nearly uniform outcome.…”

Section: Introductionmentioning

confidence: 99%

“…Etiology is eventually identified in many NORSE patients after extended workup, with sporadic or paraneoplastic autoimmune encephalitis as the dominant cause 8 . But a substantial number of cases remain cryptogenic 9 …”

Section: Introductionmentioning

confidence: 99%

“… 5 Permanent drug‐resistant epilepsy is a nearly uniform outcome. 8 We 11 , 12 , 13 , 14 and others 9 , 10 , 15 , 16 , 17 , 18 , 19 , 20 , 21 have explored an inflammatory basis for RSE in NORSE patients. Given the febrile infection‐related component of FIRES we have also postulated a maladaptive inflammatory or autoinflammatory component to pathogenesis in these patients.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Drug‐resistant seizures associated with hyperinflammatory monocytes in FIRES

Howe

Johnson

Overlee

et al. 2023

Ann Clin Transl Neurol

View full text Add to dashboard Cite

Objective: Therapeutic strategies for patients with febrile infection-related epilepsy syndrome (FIRES) are limited, ad hoc, and frequently ineffective. Based on evidence that inflammation drives pathogenesis in FIRES, we used ex vivo stimulation of peripheral blood mononuclear cells (PBMCs) to characterize the monocytic response profile before and after therapy in a child successfully treated with dexamethasone delivered intrathecally six times between hospital Day 23 and 40 at 0.25 mg/kg/dose. Methods: PBMCs were isolated from serial blood draws acquired during refractory status epilepticus (RSE) and following resolution associated with intrathecal dexamethasone therapy in a previously healthy 9-year-old male that presented with seizures following Streptococcal pharyngitis. Cells were stimulated with bacterial or viral ligands and cytokine release was measured and compared to responses in age-matched healthy control PBMCs. Levels of inflammatory factors in the blood and CSF were also measured and compared to pediatric healthy control ranges. Results: During RSE, serum levels of IL6, CXCL8, HMGB1, S100A8/A9, and CRP were significantly elevated. IL6 was elevated in CSF. Ex vivo stimulation of PBMCs collected during RSE revealed hyperinflammatory release of IL6 and CXCL8 in response to bacterial stimulation. Following intrathecal dexamethasone, RSE resolved, inflammatory levels normalized in serum and CSF, and the PBMC hyperinflammatory response renormalized. Significance: FIRES may be associated with a hyperinflammatory monocytic response to normally banal bacterial pathogens. This hyperinflammatory response may induce a profound neutrophil burden and the consequent release of factors that further exacerbate inflammation and drive neuroinflammation. Intrathecal dexamethasone may resolve RSE by resetting this inflammatory feedback loop.

show abstract

“…NORSE remains without an identifiable cause in 50-73% of the cases and typically is called cryptogenic NORSE (17)(18)(19). It is a devastating condition with a mortality rate between 10 and 30% and about two-thirds of the survivors will have functional and cognitive impairment (20).…”

Section: Introductionmentioning

confidence: 99%

Neuromodulation in new-onset refractory status epilepticus

2023

View full text Add to dashboard Cite

BackgroundNew-onset refractory status epilepticus (NORSE) and its subset of febrile infection-related epilepsy syndrome (FIRES) are devastating clinical presentations with high rates of mortality and morbidity. The recently published consensus on the treatment of these conditions includes anesthetics, antiseizure drugs, antivirals, antibiotics, and immune therapies. Despite the internationally accepted treatment, the outcome remains poor for a significant percentage of patients.MethodsWe conducted a systematic review of the use of neuromodulation techniques in the treatment of the acute phase of NORSE/FIRES using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.ResultsOur search strategy brought up 74 articles of which 15 met our inclusion criteria. A total of 20 patients were treated with neuromodulation. Thirteen cases represented FIRES and in 17 cases the NORSE remained cryptogenic. Ten had electroconvulsive therapy (ECT), seven had vagal nerve stimulation (VNS), and four had deep brain stimulation (DBS); one patient had initially VNS and later DBS. Eight patients were female and nine were children. In 17 out of 20 patients, the status epilepticus was resolved after neuromodulation, while three patients died.ConclusionNORSE can have a catastrophic course and the first treatment goal should be the fastest possible termination of status epilepticus. The data presented are limited by the small number of published cases and the variability of neuromodulation protocols used. However, they show some potential clinical benefits of early neuromodulation therapy, suggesting that these techniques could be considered within the course of FIRES/NORSE.

show abstract

New onset refractory status epilepticus: State of the art

Cited by 5 publications

References 68 publications

New-Onset Refractory Status Epilepticus Secondary to COVID-19 Infection in Adults: A Systematic Review

New-Onset Refractory Status Epilepticus Secondary to COVID-19 Infection in Adults: A Systematic Review

Drug‐resistant seizures associated with hyperinflammatory monocytes in FIRES

Neuromodulation in new-onset refractory status epilepticus

Contact Info

Product

Resources

About