Journal ArticleDOI
Characteristics of Susac syndrome: a review of all reported cases
Jan Dörr,Sarah Krautwald,Brigitte Wildemann,Sven Jarius,Marius Ringelstein,Thomas Duning,Orhan Aktas,Erich Bernd Ringelstein,Friedemann Paul,Ilka Kleffner +9 more
TLDR
From all 304 cases of Susac syndrome that have been published worldwide, which were identified following a literature search with predefined search, inclusion and exclusion criteria, an overview of demographic, clinical and diagnostic data is presented, providing a reliable basis for current understanding of this rare disease.Abstract:
In Susac syndrome, occlusions of microvessels—presumed to be mediated by an autoimmune response to an as yet unknown antigen—lead to a characteristic clinical triad of CNS dysfunction, branch retinal artery occlusions, and sensorineural hearing impairment. Susac syndrome is considered a rare but important differential diagnosis in numerous neurological, psychiatric, ophthalmological, and ear, nose and throat disorders. Improved understanding of this disorder is crucial, therefore, to ensure that patients receive appropriate treatment and care. Current knowledge on Susac syndrome is largely based on reports of single patients, small case series, and nonsystematic reviews. The aim of this Review is to extend these previous, primarily anecdotal findings by compiling data from all 304 cases of Susac syndrome that have been published worldwide, which were identified following a literature search with predefined search, inclusion and exclusion criteria. From this data, we present an overview of demographic, clinical and diagnostic data on Susac syndrome, providing a reliable basis for our current understanding of this rare disease. Where possible, we make recommendations for clinical diagnosis, differential diagnosis, and management of patients with suspected Susac syndrome.read more
Citations
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A clinical approach to diagnosis of autoimmune encephalitis
Francesc Graus,Maarten J. Titulaer,Ramani Balu,Susanne M. Benseler,Christian G. Bien,Tania Cellucci,Irene Cortese,Russell C. Dale,Jeffrey M. Gelfand,Michael D. Geschwind,Carol A. Glaser,Jérôme Honnorat,Romana Höftberger,Takahiro Iizuka,Sarosh R. Irani,Eric Lancaster,Frank Leypoldt,Harald Prüss,Alexander Rae-Grant,Markus Reindl,Myrna R. Rosenfeld,Kevin Rostasy,Albert Saiz,Arun Venkatesan,Angela Vincent,Klaus Peter Wandinger,Patrick Waters,J. Dalmau,J. Dalmau +28 more
TL;DR: Through logical differential diagnosis, levels of evidence for autoimmune encephalitis (possible, probable, or definite) are achieved, which can lead to prompt immunotherapy.
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Diagnostic and therapeutic challenges.
TL;DR: This case is submitted by Drs.
Journal ArticleDOI
Diagnostic criteria for Susac syndrome
Ilka Kleffner,Jan Dörr,Marius Ringelstein,Catharina C. Gross,Yvonne Böckenfeld,Wolfram Schwindt,Benedikt Sundermann,Hubertus Lohmann,Heike Wersching,Julia Promesberger,Natascha von Königsmarck,Anne F. Alex,Rainer Guthoff,Catharina J.M. Frijns,L. Jaap Kappelle,Sven Jarius,Brigitte Wildemann,Orhan Aktas,Friedemann Paul,Heinz Wiendl,Thomas Duning +20 more
TL;DR: Integrating the clinical presentation and paraclinical findings, the proposed formal criteria and recommend a diagnostic workup to facilitate the diagnosis of Susac syndrome are proposed and surmise that more patients could have been diagnosed.
Journal ArticleDOI
The current role of MRI in differentiating multiple sclerosis from its imaging mimics
Ruth Geraldes,Olga Ciccarelli,Frederik Barkhof,Nicola De Stefano,Christian Enzinger,Massimo Filippi,Monika Hofer,Friedemann Paul,Paolo Preziosa,Alex Rovira,Gabriele C. DeLuca,Ludwig Kappos,Tarek A. Yousry,Franz Fazekas,Jette L. Frederiksen,Claudio Gasperini,Jaume Sastre-Garriga,Nikos Evangelou,Jacqueline Palace +18 more
TL;DR: MAGNIMS updates the imaging features that differentiate the most common mimics of MS, particularly age-related cerebrovascular disease and neuromyelitis optica, from MS itself and provides a pragmatic summary of the clinically useful MRI features that distinguish MS from its mimics.
Journal ArticleDOI
Retinal and Ophthalmic Artery Occlusions Preferred Practice Pattern
Timothy W. Olsen,Jose S. Pulido,James C. Folk,Leslie Hyman,Christina J. Flaxel,Ron A. Adelman +5 more
TL;DR: This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record.
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Diagnostic criteria for Susac syndrome
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