New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome.

PURPOSE OF REVIEW: The concept and understanding of new-onset refractory status epilepticus (NORSE), and its subtype with prior fever known as febrile infection-related epilepsy syndrome (FIRES) have evolved in the recent past. This review aims to summarize the recent developments in the pathophysiology, diagnosis and management of these challenging conditions. RECENT FINDINGS: NORSE and FIRES can have many different causes. Although the list of possible causes is still growing, they mostly fall in the categories of autoimmune encephalitis and genetic disorders. However, despite extensive investigations, most cases of NORSE and FIRES remain cryptogenic. Recent studies have pointed towards the key role of autoinflammation as a unifying pathophysiological mechanism in these cases. These findings also support the use of immunomodulatory treatment in this setting. Consensus recommendations on the management of NORSE and FIRES have recently been published. SUMMARY: NORSE and FIRES remain challenging conditions to diagnose and treat. Recent findings from clinical and basic research and new recommendations, reviewed in this article, contribute to an emerging framework for management and future research.

Infant with Loeys-Dietz syndrome treated for febrile status epilepticus with COVID-19 infection: first reported case of febrile status epilepticus and focal seizures in a patient with Loeys-Dietz syndrome and review of literature.

Loeys-Dietz syndrome (LDS) is a rare, autosomal dominant multisystem disorder that is caused by mutations of transforming growth factor-ß receptors. Mutations in SMAD3 and TGFB3 have been recently reported.LDS is characterised by the triad of arterial tortuosity, hypertelorism and a bifid uvula or cleft palate among other cardiovascular, craniofacial and orthopaedic manifestations. Patients with LDS show clinical and genetic variability and there is a significant risk of reduced life expectancy due to widespread arterial involvement, aortic root dilation, aneurysms and an aggressive vascular course. Thus early genetic testing is warranted if clinical signs and history are suggestive of this potentially catastrophic disorder.LDS predisposes patients to aortic aneurysms and early death due to vascular malformations, but neurological emergencies, such as seizures and febrile status epilepticus, have not been reported.Febrile status epilepticus is the most common neurological emergency in childhood. Neurological manifestations of COVID-19 in the paediatric population are not as well described in medical literature.To the best of our knowledge, this is the first reported case of febrile status epilepticus with COVID-19 infection in an infant with LDS. Our patient had focal epileptiform activity emanating over the left posterior hemisphere, which evolved into an electrographic seizure on video EEG. Such patients have a heightened risk of epilepsy in the future, and this occurrence is consistent with a diagnosis of focal epilepsy. Neurological complications such as epilepsy and status epilepticus in a patient with LDS have never been reported before.A brief review of literature is also given here.

Status epilepticus during the COVID-19 pandemic in Cologne, Germany: data from a retrospective, multicentre registry.

BACKGROUND: The "coronavirus disease 2019" (COVID-19) pandemic, caused by the "severe-acute-respiratory-syndrome-coronavirus 2" (SARS-CoV-2), challenges healthcare systems worldwide and impacts not only COVID-19 patients but also other emergencies. To date, data are scarce on the extent to which the COVID-19 pandemic impacted status epilepticus (SE) and its treatment. OBJECTIVE: To assess the influence of the COVID-19 pandemic on the incidence, management and outcome of SE patients. STUDY DESIGN: This is a retrospective, multicentre trial, approved by the University of Cologne (21-1443-retro). METHODS: All SE patients from the urban area of Cologne transmitted to all acute neurological departments in Cologne between 03/2019 and 02/2021 were retrospectively analysed and assessed for patient characteristics, SE characteristics, management, and outcome in the first pandemic year compared to the last pre-pandemic year. RESULTS: 157 pre-pandemic (03/2019-02/2020) and 171 pandemic (from 03/2020 to 02/2021) SE patients were included in the analyses. Acute SARS-CoV-2 infections were rarely detected. Patient characteristics, management, and outcome did not reveal significant groupwise differences. In contrast, regarding prehospital management, a prolonged patient transfer to the hospital and variations in SE aetiologies compared to the last pre-pandemic year were observed with less chronic vascular and more cryptogenic and anoxic SE cases. No infections with SARS-CoV-2 occurred during inpatient stays. CONCLUSIONS: SARS-CoV-2 infections did not directly affect SE patients, but the transfer of SE patients to emergency departments was delayed. Interestingly, SE aetiology rates shifted, which warrants further exploration. Fears of contracting an in-hospital SARS-CoV-2-infection were unfounded due to consequent containment measures.

The effects of COVID-19 measures on the hospitalization of patients with epilepsy and status epilepticus in Thailand: An interrupted time series analysis.

OBJECTIVE: To investigate the effect of Coronavirus disease 2019 (COVID-19) measures on the hospitalization of patients with epilepsy and status epilepticus (SE). METHODS: This interrupted time series design included data from the Thai Universal Coverage Scheme electronic database between January 2017 and September 2020. The monthly hospitalization rate of epilepsy and SE was calculated by the number of hospitalizations divided by the midyear population. Segmented regression fitted by ordinary least squares (OLS) was used to detect the immediate and overtime effects of COVID-19 measures on the hospitalization rate. RESULTS: During January 2017 and September 2020, the numbers of epilepsy and SE patients admitted to the hospital were 129 402 and 15 547 episodes, respectively. The monthly trend of the hospitalization rate in epilepsy decreased immediately after the COVID-19 measure (0.739 per 100 000 population [95% CI: 0.219 to 1.260]). In particular, the number of children declined to 1.178 per 100 000 population, and the number of elderly individuals dropped to 0.467 per 100 000 population, while there was a nonstatistically significant change in SE. SIGNIFICANCE: COVID-19 measures reduced the hospital rate in epilepsy, particularly in children and adults. However, there was no change in SE patients.

Evaluation and Treatment of Seizures and Epilepsy During the COVID-19 Pandemic.

PURPOSE OF REVIEW: Seizures, including status epilepticus, have been reported in association with acute COVID-19 infection. People with epilepsy (PWE) have suffered from seizure exacerbations during the pandemic. This article reviews the data for clinical and electrographic seizures associated with COVID-19, technical EEG considerations for reducing risk of transmission, and factors contributing to seizure exacerbations in PWE as well as strategies to address this issue. RECENT FINDINGS: An increasing number of studies of larger cohorts, accounting for a variety of variables and often utilizing EEG with standardized terminology, are assessing the prevalence of seizures in hospitalized patients with acute COVID-19 infections, and gaining insight into the prevalence of seizures and their effect on outcomes. Additionally, recent studies are evaluating the effect of the pandemic on PWE, barriers faced, and the usefulness of telehealth. Although there is still much to learn regarding COVID-19, current studies help in assessing the risk of seizures, guiding EEG utilization, and optimizing the use of telehealth during the pandemic.

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

BACKGROUND AND PURPOSE: New-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. METHODS: A 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. RESULTS: Four 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. CONCLUSIONS: Far 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.

Management of Neurological Emergencies in Children: An Updated Overview.

Neurological emergencies account for about one-third of the highest severity codes attributed in emergency pediatric departments. About 75% of children with acute neurological symptoms presents with seizures, headache, or other paroxysmal events. Life-threatening conditions involve a minor proportion of patients (e.g., less than 15% of children with headache and less than 5% of children with febrile seizures). This review highlights updated insights about clinical features, diagnostic workup, and therapeutic management of pediatric neurological emergencies. Particularly, details will be provided about the most recent insights about headache, febrile seizures, status epilepticus, altered levels of consciousness, acute motor impairment, acute movement disorders, and functional disorders, as well as the role of diagnostic tools (e.g., neuroimaging, lumbar puncture, and electroencephalography), in the emergency setting. Moreover, the impact of the current novel coronavirus disease2019 (COVID-19) pandemic on the evaluation of pediatric neurologic emergencies will also be analyzed.

New-onset refractory status epilepticus following the ChAdOx1 nCoV-19 vaccine.

Coronavirus is a novel human pathogen causing fulminant respiratory syndrome (COVID-19). Developing an effective and reliable vaccine was emergently pursued to control the dramatic spread of the global pandemic. The standard stages for vaccine development were unprecedentedly accelerated over a few months. We report a case of new-onset refractory status epilepticus (NORSE) after receiving the first dose of the ChAdOx1 nCoV-19 vaccine. We attribute the occurrence of NORSE to the vaccine due to the temporal relationship and the lack of risk factors for epilepsy in the patient. This report adds to the literature a possible rare side effect of a COVID-19 vaccine and contributes to the extremely limited literature on potential neurological side effects of viral vector vaccines. Healthcare providers should be aware of the possibility of post-vaccination epilepsy. The patient had recurrent seizures that were refractory to conventional antiepileptic drug therapy with a dramatic response to immunotherapy with pulse steroids and plasmapheresis. This likely reflects an underlying autoimmune mechanism in the genesis of post-vaccination generalized seizures without fever. Further research is needed to probe and study the exact mechanism at a more molecular level.