title: Diagnostic criteria for Susac syndrome.
abstract: BACKGROUND
  Susac syndrome is characterised by the triad of encephalopathy with or without focal neurological signs, branch retinal artery occlusions and hearing loss. Establishment of the diagnosis is often delayed because the triad is complete only in a minority of patients at disease onset. This leads to a critical delay in the initiation of appropriate treatment. Our objective was to establish criteria for diagnosis of either definite or probable Susac syndrome.
  
  
  METHODS
  The establishment of diagnostic criteria was based on the following three steps: (1) Definition of a reference group of 32 patients with an unambiguous diagnosis of Susac syndrome as assessed by all interdisciplinary experts of the European Susac Consortium (EuSaC) team (EuSaC cohort); (2) selection of diagnostic criteria, based on common clinical and paraclinical findings in the EuSaC cohort and on a review of the literature; and (3) validation of the proposed criteria in the previously published cohort of all Susac cases reported until 2012.
  
  
  RESULTS
  Integrating the clinical presentation and paraclinical findings, we propose formal criteria and recommend a diagnostic workup to facilitate the diagnosis of Susac syndrome. More than 90% of the cases in the literature fulfilled the proposed criteria for probable or definite Susac syndrome. We surmise that more patients could have been diagnosed with the recommended diagnostic workup.
  
  
  CONCLUSIONS
  We propose diagnostic criteria for Susac syndrome that may help both experts and physicians not familiar with Susac syndrome to make a correct diagnosis and to prevent delayed treatment initiation.
authors.affiliation: Department of Neurology, University Hospital of Münster, Münster, Germany.
authors.forename: Ilka
authors.initials: I
authors.lastname: Kleffner
authors.affiliation: NeuroCure Clinical Research Center, Charité-Universitätsmedizin Berlin, Berlin, Germany.
authors.forename: Jan
authors.initials: J
authors.lastname: Dörr
authors.affiliation: Department of Neurology, Medical Faculty, Heinrich-Heine-University, Düsseldorf, Germany.
authors.forename: Marius
authors.initials: M
authors.lastname: Ringelstein
authors.affiliation: Department of Neurology, University Hospital of Münster, Münster, Germany.
authors.forename: Catharina C
authors.initials: CC
authors.lastname: Gross
authors.affiliation: Department of Otorhinolaryngology, Head and Neck Surgery, University of Münster, Münster, Germany.
authors.forename: Yvonne
authors.initials: Y
authors.lastname: Böckenfeld
authors.affiliation: Department of Clinical Radiology, University of Münster, Münster, Germany.
authors.forename: Wolfram
authors.initials: W
authors.lastname: Schwindt
authors.affiliation: Department of Clinical Radiology, University of Münster, Münster, Germany.
authors.forename: Benedikt
authors.identifier: http://orcid.org/0000-0003-4390-3464
authors.initials: B
authors.lastname: Sundermann
authors.affiliation: Department of Neurology, University Hospital of Münster, Münster, Germany.
authors.forename: Hubertus
authors.initials: H
authors.lastname: Lohmann
authors.affiliation: Institute of Epidemiology and Social Medicine, University of Münster, Münster, Germany.
authors.forename: Heike
authors.initials: H
authors.lastname: Wersching
authors.affiliation: Department of Ophthalmology, University of Münster, Münster, Germany.
authors.forename: Julia
authors.initials: J
authors.lastname: Promesberger
authors.affiliation: Department of Ophthalmology, University of Münster, Münster, Germany.
authors.forename: Natascha
authors.initials: N
authors.lastname: von Königsmarck
authors.affiliation: Department of Ophthalmology, University of Münster, Münster, Germany.
authors.forename: Anne
authors.initials: A
authors.lastname: Alex
authors.affiliation: Department of Ophthalmology, Medical Faculty, Heinrich-Heine-University, Düsseldorf, Germany.
authors.forename: Rainer
authors.initials: R
authors.lastname: Guthoff
authors.affiliation: Department of Neurology, Brain Centre Rudolph Magnus, University Medical Centre Utrecht, Utrecht, The Netherlands.
authors.forename: Catharina J M
authors.initials: CJ
authors.lastname: Frijns
authors.affiliation: Department of Neurology, Brain Centre Rudolph Magnus, University Medical Centre Utrecht, Utrecht, The Netherlands.
authors.forename: L Jaap
authors.initials: LJ
authors.lastname: Kappelle
authors.affiliation: Molecular Neuroimmunology Working Group, Department of Neurology, University of Heidelberg, Germany.
authors.forename: Sven
authors.initials: S
authors.lastname: Jarius
authors.affiliation: Molecular Neuroimmunology Working Group, Department of Neurology, University of Heidelberg, Germany.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Wildemann
authors.affiliation: Department of Neurology, Medical Faculty, Heinrich-Heine-University, Düsseldorf, Germany.
authors.forename: Orhan
authors.initials: O
authors.lastname: Aktas
authors.affiliation: NeuroCure Clinical Research Center, Charité-Universitätsmedizin Berlin, Berlin, Germany.|Department of Neurology, Clinical and Experimental Multiple Sclerosis Research Center, Charité-Universitätsmedizin Berlin, Berlin, Germany.|Experimental and Clinical Research Center, Max Delbrueck Center for Molecular Medicine and Charité-Universitätsmedizin Berlin, Berlin, Germany.
authors.forename: Friedemann
authors.initials: F
authors.lastname: Paul
authors.affiliation: Department of Neurology, University Hospital of Münster, Münster, Germany.
authors.forename: Heinz
authors.initials: H
authors.lastname: Wiendl
authors.affiliation: Department of Neurology, University Hospital of Münster, Münster, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Duning
mesh_terms: D000293:Adolescent; D000328:Adult; D015331:Cohort Studies; D057210:Delayed Diagnosis; D003937:Diagnosis, Differential; D061345:Early Medical Intervention; D005260:Female; D019983:Guideline Adherence; D006801:Humans; D033183:Interdisciplinary Communication; D000070516:Intersectoral Collaboration; D008297:Male; D008875:Middle Aged; D012016:Reference Values; D055955:Susac Syndrome / Q000175:diagnosis* / Q000628:therapy; D055815:Young Adult