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It refers to a patient without active epilepsy or other pre\u2010existing 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\u2010related conditions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A systematic search was carried out for clinical trials, observational studies, case series and case reports including patients who presented with NORSE, febrile\u2010infection\u2010related epilepsy syndrome or the infantile hemiconvulsion\u2010hemiplegia and epilepsy syndrome.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Four hundred and fifty records were initially identified, of which 197 were included in the review. The selected studies were retrospective case\u2013control (<jats:italic>n<\/jats:italic>\u00a0=\u00a011), case series (<jats:italic>n<\/jats:italic>\u00a0=\u00a083) and case reports (<jats:italic>n<\/jats:italic>\u00a0=\u00a0103) and overall described 1334 patients both of paediatric and adult age. Aetiology remains unexplained in about half of the cases, representing the so\u2010called \u2018cryptogenic NORSE\u2019. Amongst adult patients without cryptogenic NORSE, the most often identified cause is autoimmune encephalitis, either non\u2010paraneoplastic or paraneoplastic. Infections are the prevalent aetiology of paediatric non\u2010cryptogenic NORSE. Genetic and congenital disorders can have a causative role in NORSE, and toxic, vascular and degenerative conditions have also been described.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>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\u2010up and guide therapeutic approaches should be implemented. Global cooperation and multicentre research represent priorities to improve the understanding of NORSE.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.15149","type":"journal-article","created":{"date-parts":[[2021,10,20]],"date-time":"2021-10-20T18:44:07Z","timestamp":1634755447000},"page":"626-647","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":66,"title":["Unraveling the enigma of new\u2010onset refractory status epilepticus: a systematic review of aetiologies"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8748-0083","authenticated-orcid":false,"given":"Simona","family":"Lattanzi","sequence":"first","affiliation":[{"name":"Neurological Clinic Department of Experimental and Clinical Medicine Marche Polytechnic University Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Leitinger","sequence":"additional","affiliation":[{"name":"Department of Neurology Christian Doppler University Hospital Center for Cognitive Neuroscience Paracelsus Medical University Salzburg Austria"},{"name":"Neuroscience Institute Christian Doppler University Hospital Center for Cognitive Neuroscience Paracelsus Medical University Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1215-9583","authenticated-orcid":false,"given":"Chiara","family":"Rocchi","sequence":"additional","affiliation":[{"name":"Neurological Clinic Department of Experimental and Clinical Medicine Marche Polytechnic University Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sergio","family":"Salvemini","sequence":"additional","affiliation":[{"name":"Neurological Clinic Department of Experimental and Clinical Medicine Marche Polytechnic University Ancona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4403-6342","authenticated-orcid":false,"given":"Sara","family":"Matricardi","sequence":"additional","affiliation":[{"name":"Department of Child Neuropsychiatry Children's Hospital \u2018G. 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