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

Autor: Lattanzi S; Neurological Clinic, Department of Experimental and Clinical Medicine, Marche Polytechnic University, Ancona, Italy., Leitinger M; Department of Neurology, Christian Doppler University Hospital, Center for Cognitive Neuroscience, Paracelsus Medical University, Salzburg, Austria.; Neuroscience Institute, Christian Doppler University Hospital, Center for Cognitive Neuroscience, Paracelsus Medical University, Salzburg, Austria., Rocchi C; Neurological Clinic, Department of Experimental and Clinical Medicine, Marche Polytechnic University, Ancona, Italy., Salvemini S; Neurological Clinic, Department of Experimental and Clinical Medicine, Marche Polytechnic University, Ancona, Italy., Matricardi S; Department of Child Neuropsychiatry, Children's Hospital 'G. Salesi', Ancona, Italy., Brigo F; Department of Neuroscience, Biomedicine and Movement Science, University of Verona, Verona, Italy.; Division of Neurology, 'Franz Tappeiner' Hospital, Merano, BZ, Italy., Meletti S; Neurology Unit, OCB Hospital, AOU Modena, Modena, Italy.; Department of Biomedical, Metabolic and Neural Science, Center for Neuroscience and Neurotechnology, University of Modena and Reggio Emilia, Modena, Italy., Trinka E; Department of Neurology, Christian Doppler University Hospital, Center for Cognitive Neuroscience, Paracelsus Medical University, Salzburg, Austria.; Neuroscience Institute, Christian Doppler University Hospital, Center for Cognitive Neuroscience, Paracelsus Medical University, Salzburg, Austria.; Public Health, Health Services Research and HTA, Medical Informatics and Technology, University for Health Sciences, Hall i.T, Austria.
Jazyk: angličtina
Zdroj: European journal of neurology [Eur J Neurol] 2022 Feb; Vol. 29 (2), pp. 626-647. Date of Electronic Publication: 2021 Nov 02.
DOI: 10.1111/ene.15149
Abstrakt: 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.
(© 2021 The Authors. European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)
Databáze: MEDLINE
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