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J Neurol Neurosurg Psychiatry. 2016 Dec;87(12):1287-1295. doi: 10.1136/jnnp-2016-314295. Epub 2016 Oct 25.

Diagnostic criteria for Susac syndrome.

Author information

1
Department of Neurology, University Hospital of Münster, Münster, Germany.
2
NeuroCure Clinical Research Center, Charité-Universitätsmedizin Berlin, Berlin, Germany.
3
Department of Neurology, Medical Faculty, Heinrich-Heine-University, Düsseldorf, Germany.
4
Department of Otorhinolaryngology, Head and Neck Surgery, University of Münster, Münster, Germany.
5
Department of Clinical Radiology, University of Münster, Münster, Germany.
6
Institute of Epidemiology and Social Medicine, University of Münster, Münster, Germany.
7
Department of Ophthalmology, University of Münster, Münster, Germany.
8
Department of Ophthalmology, Medical Faculty, Heinrich-Heine-University, Düsseldorf, Germany.
9
Department of Neurology, Brain Centre Rudolph Magnus, University Medical Centre Utrecht, Utrecht, The Netherlands.
10
Molecular Neuroimmunology Working Group, Department of Neurology, University of Heidelberg, Germany.
11
Department of Neurology, Clinical and Experimental Multiple Sclerosis Research Center, Charité-Universitätsmedizin Berlin, Berlin, Germany.
12
Experimental and Clinical Research Center, Max Delbrueck Center for Molecular Medicine and Charité-Universitätsmedizin Berlin, Berlin, Germany.

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.

METHOD:

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.

PMID:
28103199
DOI:
10.1136/jnnp-2016-314295
[Indexed for MEDLINE]

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