Unraveling the enigma of new-onset refractory status epilepticus: a systematic review of aetiologies.
Eur J Neurol
; 29(2): 626-647, 2022 02.
Article
in English
| MEDLINE | ID: covidwho-1518031
ABSTRACT
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.Keywords
Full text:
Available
Collection:
International databases
Database:
MEDLINE
Main subject:
Status Epilepticus
/
Encephalitis
/
Drug Resistant Epilepsy
/
Epileptic Syndromes
Type of study:
Diagnostic study
/
Etiology study
/
Observational study
/
Prognostic study
/
Reviews
/
Systematic review/Meta Analysis
Topics:
Long Covid
Limits:
Adult
/
Child
/
Humans
Language:
English
Journal:
Eur J Neurol
Journal subject:
Neurology
Year:
2022
Document Type:
Article
Affiliation country:
Ene.15149
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