Atypical clinical and novel radiological findings in Susac syndrome: Experience from a large monocentric cohort

Cathérine Dekeyser; Alexander Vanhoorne; Dimitri Hemelsoet; Liesbeth Van Hijfte; Julie De Zaeytijd; Veroniek Van Driessche; Helen Van Hoecke; Marijke Miatton; Tineke Van Vrekhem; Leen Maes; Guy Laureys

doi:10.1016/j.jneuroim.2023.578032

Atypical clinical and novel radiological findings in Susac syndrome: Experience from a large monocentric cohort

J Neuroimmunol. 2023 Mar 15:376:578032. doi: 10.1016/j.jneuroim.2023.578032. Epub 2023 Jan 21.

Authors

Affiliations

¹ Department of Neurology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address: [email protected].
² Department of Neurology, AZ Groeninge, President Kennedylaan 4, Kortrijk, Belgium. Electronic address: [email protected].
³ Department of Neurology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address: [email protected].
⁴ Department of Neurology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address: [email protected].
⁵ Department of Ophthalmology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address: [email protected].
⁶ Department of Radiology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address: [email protected].
⁷ Department of Otorhinolaryngology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address: [email protected].
⁸ Department of Neurology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address: [email protected].
⁹ Department of Neurology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address: [email protected].
¹⁰ Department of Otorhinolaryngology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium; Department of Rehabilitation Sciences, Ghent University, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address: [email protected].
¹¹ Department of Neurology, Ghent University Hospital, Corneel Heymanslaan 10, Ghent, Belgium. Electronic address: [email protected].

PMID: 36736020
DOI: 10.1016/j.jneuroim.2023.578032

Abstract

Susac syndrome (SuS) is a rare immune-mediated endotheliopathy that affects the brain, retina and inner ear and is characterised by the variable clinical triad of encephalopathy, visual and vestibulocochlear dysfunction. Here, we present clinical and paraclinical data of 19 SuS patients followed at Ghent University Hospital and highlight some atypical clinical and novel radiological findings. Our findings suggest that spinal involvement expands the clinical phenotype of SuS. We further introduce dark blood sequences as a more sensitive technique to detect radiological disease activity in SuS. Our data add to the current understanding of the diagnosis, monitoring and treatment of SuS.

Keywords: Branch retinal artery occlusion; Dark blood imaging; Endotheliopathy; Sensorineural hearing loss; Spinal cord symptoms; Susac syndrome.

MeSH terms

Brain
Brain Diseases*
Humans
Magnetic Resonance Imaging
Retina
Susac Syndrome* / diagnosis