New-onset refractory status epilepticus (NORSE) denotes a heterogeneous group of rare disorders and diseases that are characterized by de novo onset of refractory status epilepticus. To date, no acute or active structural, toxic, or metabolic cause has been identified for this devastating condition. Though an etiology can be found in some instances, in about 80% of patients the underlying cause remains unknown. The current assumption regarding the refractoriness of seizures in this condition is that neuroinflammation, triggered by immunological mechanisms, causes an escalating cycle of seizures which in turn facilitate the inflammation. Despite some studies being published on NORSE, or specific subgroups such as febrile infection-related epilepsy syndrome (FIRES), no high-level evidence exists regarding its diagnosis or treatment. This is troublesome as patients have a very poor prognosis, with estimated mortality rates of 10-30% and severe sequelae including neuropsychological impairments and refractory epilepsy in most survivors.
To date, roughly 200 studies have been published on NORSE, but most of the reports concern single cases or retrospective cohorts, making it difficult to draw conclusions regarding the best way of diagnosing and treating affected patients. As we have seen extensive development of specialized clinical facilities forming collaborations and international networks within the last decades as well as an increased attention and shifting focus onto rare diseases and personalized medicine, it can be expected that new evidence has emerged within the last years on rare diseases and conditions such as NORSE. In addition, the establishment of networks between centers and countries has led to an overall increase in the availability of advanced technological and analytical tools aiding diagnosis and research on causes and mechanisms of rare diseases, suggesting that the detail and level of workup in these patients has increased even more. Given these recent developments, this research topic aims at collecting new evidence on any aspect of NORSE and its subgroups, in order to enable the development of guidelines on its diagnosis and treatment.
We are interested in any of the following types of high-quality manuscripts: original research, case series, systematic review, and meta-analysis, which are addressing the issue from a fundamental research or clinical perspective. We are interested in research studies investigating the following aspects of NORSE or its subgroups:
· neuronal mechanisms of immunological or inflammatory processes due to NORSE
· etiological factors of NORSE
· advances in the diagnosis of NORSE with special emphasis on practicability
· advances in treatment (pharmacologic or neuromodulatory) approaches and targets
· biomarkers for disease progression, treatments success, and long-term effects of NORSE
New-onset refractory status epilepticus (NORSE) denotes a heterogeneous group of rare disorders and diseases that are characterized by de novo onset of refractory status epilepticus. To date, no acute or active structural, toxic, or metabolic cause has been identified for this devastating condition. Though an etiology can be found in some instances, in about 80% of patients the underlying cause remains unknown. The current assumption regarding the refractoriness of seizures in this condition is that neuroinflammation, triggered by immunological mechanisms, causes an escalating cycle of seizures which in turn facilitate the inflammation. Despite some studies being published on NORSE, or specific subgroups such as febrile infection-related epilepsy syndrome (FIRES), no high-level evidence exists regarding its diagnosis or treatment. This is troublesome as patients have a very poor prognosis, with estimated mortality rates of 10-30% and severe sequelae including neuropsychological impairments and refractory epilepsy in most survivors.
To date, roughly 200 studies have been published on NORSE, but most of the reports concern single cases or retrospective cohorts, making it difficult to draw conclusions regarding the best way of diagnosing and treating affected patients. As we have seen extensive development of specialized clinical facilities forming collaborations and international networks within the last decades as well as an increased attention and shifting focus onto rare diseases and personalized medicine, it can be expected that new evidence has emerged within the last years on rare diseases and conditions such as NORSE. In addition, the establishment of networks between centers and countries has led to an overall increase in the availability of advanced technological and analytical tools aiding diagnosis and research on causes and mechanisms of rare diseases, suggesting that the detail and level of workup in these patients has increased even more. Given these recent developments, this research topic aims at collecting new evidence on any aspect of NORSE and its subgroups, in order to enable the development of guidelines on its diagnosis and treatment.
We are interested in any of the following types of high-quality manuscripts: original research, case series, systematic review, and meta-analysis, which are addressing the issue from a fundamental research or clinical perspective. We are interested in research studies investigating the following aspects of NORSE or its subgroups:
· neuronal mechanisms of immunological or inflammatory processes due to NORSE
· etiological factors of NORSE
· advances in the diagnosis of NORSE with special emphasis on practicability
· advances in treatment (pharmacologic or neuromodulatory) approaches and targets
· biomarkers for disease progression, treatments success, and long-term effects of NORSE