Literature DB >> 31278469

[Susac syndrome-interdisciplinary tracking of the chameleon: two different case reports].

D Schelenz1,2, I Kleffner3, N Tsiampalis1,4, H Burkhard Dick1, J Rehrmann5.   

Abstract

BACKGROUND: The diagnosis of Susac syndrome, a presumed autoimmune retinocochleocerebral microvasculopathy, is extremely complex. At the onset of this orphan disease patients can present with an incomplete clinical triad consisting of sensorineural hearing loss, visual loss because of retinal ischemia and diverse neurological symptoms. In terms of the pathophysiology, it is assumed that the vascular endothelial cells swell, occlude the lumina of the vessels and consequently cause ischemia in the surrounding tissue. Due to the wide range of symptoms it is extremely challenging to establish a correct diagnosis. Susac syndrome should be considered as an important differential diagnosis from other neurological, ophthalmological, psychological and otorhinolaryngological diseases. CASE REPORT: This report presents two different courses of the disease in patients with Susac's syndrome. The first case of a 46-year-old woman, with previously confirmed Susac's syndrome, describes the treatment adjustment and monitoring. The second case of a 30-year-old woman shows the establishment of the initial diagnosis. DISCUSSION: The two reported cases of Susac's syndrome show the multifaceted range of clinical findings and courses of the disease. Furthermore, the diagnostic and therapeutic options are discussed with respect to the current literature. Diagnostic criteria already published by the European Susac Consortium and a good interdisciplinary collaboration enable a diagnosis as early as possible, which is essential for avoiding delayed treatment and reducing morbidity.

Entities:  

Keywords:  Autoimmune vasculopathy; Branch retinal artery occlusion; Corpus callosum; Microangiopathy; Susac syndrome

Mesh:

Year:  2020        PMID: 31278469     DOI: 10.1007/s00347-019-0926-y

Source DB:  PubMed          Journal:  Ophthalmologe        ISSN: 0941-293X            Impact factor:   1.059


  15 in total

1.  Retinal and optic nerve head pathology in Susac's syndrome.

Authors:  D Scott McLeod; Howard S Ying; Colin A McLeod; Rhonda Grebe; Martin Lubow; John O Susac; Gerard A Lutty
Journal:  Ophthalmology       Date:  2011-03       Impact factor: 12.079

2.  Susac's Syndrome--update.

Authors:  Robert Rennebohm; John O Susac; Robert A Egan; Robert B Daroff
Journal:  J Neurol Sci       Date:  2010-09-19       Impact factor: 3.181

Review 3.  [Susac syndrome: an interdisciplinary challenge].

Authors:  J Dörr; S Jarius; B Wildemann; E B Ringelstein; W Schwindt; M Deppe; K P Wandinger; J Promesberger; F Paul; I Kleffner
Journal:  Nervenarzt       Date:  2011-10       Impact factor: 1.214

4.  Exacerbation of Susac syndrome retinopathy by interferon Beta-1a.

Authors:  Philip W Laird; Nancy J Newman; Steven Yeh
Journal:  Arch Ophthalmol       Date:  2012-06

Review 5.  Characteristics of Susac syndrome: a review of all reported cases.

Authors:  Jan Dörr; Sarah Krautwald; Brigitte Wildemann; Sven Jarius; Marius Ringelstein; Thomas Duning; Orhan Aktas; Erich Bernd Ringelstein; Friedemann Paul; Ilka Kleffner
Journal:  Nat Rev Neurol       Date:  2013-04-30       Impact factor: 42.937

6.  Brain histopathology in three cases of Susac's syndrome: implications for lesion pathogenesis and treatment.

Authors:  Todd A Hardy; Billy O'Brien; Natasha Gerbis; Michael H Barnett; Stephen W Reddel; Janice Brewer; Geoffrey K Herkes; Paul Silberstein; Roger J Garsia; John D G Watson; Ruta Gupta; John D E Parratt; Michael E Buckland
Journal:  J Neurol Neurosurg Psychiatry       Date:  2014-08-28       Impact factor: 10.154

Review 7.  Update on Susac syndrome: new insights in brain and retinal imaging and treatment options.

Authors:  Jan Dörr; Marius Ringelstein; Thomas Duning; Ilka Kleffner
Journal:  J Alzheimers Dis       Date:  2014       Impact factor: 4.472

8.  Gass plaques and fluorescein leakage in Susac Syndrome.

Authors:  Robert A Egan; William L Hills; John O Susac
Journal:  J Neurol Sci       Date:  2010-09-29       Impact factor: 3.181

9.  Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography.

Authors:  Marius Ringelstein; Philipp Albrecht; Ilka Kleffner; Björn Bühn; Jens Harmel; Ann-Kristin Müller; David Finis; Rainer Guthoff; Richard Bergholz; Thomas Duning; Markus Krämer; Friedemann Paul; Alexander Brandt; Timm Oberwahrenbrock; Janine Mikolajczak; Brigitte Wildemann; Sven Jarius; Hans-Peter Hartung; Orhan Aktas; Jan Dörr
Journal:  Neurology       Date:  2015-07-22       Impact factor: 9.910

10.  Patterns of retinal damage facilitate differential diagnosis between Susac syndrome and MS.

Authors:  Alexander U Brandt; Hanna Zimmermann; Falko Kaufhold; Julia Promesberger; Sven Schippling; David Finis; Orhan Aktas; Christian Geis; Marius Ringelstein; E Bernd Ringelstein; Hans-Peter Hartung; Friedemann Paul; Ilka Kleffner; Jan Dörr
Journal:  PLoS One       Date:  2012-06-11       Impact factor: 3.240

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