Renate Engisch1, David S Titelbaum2, Lara Chilver-Stainer3, Frauke Kellner-Weldon4. 1. Institute of Diagnostic and Interventional Neuroradiology, Bern University Hospital, Bern, Switzerland renate.engisch@insel.ch. 2. Shields Healthcare, Brockton, MA, USA. 3. Department of Neurology, Bern University Hospital, Bern, Switzerland. 4. Institute of Diagnostic and Interventional Neuroradiology, Bern University Hospital, Bern, Switzerland.
Abstract
BACKGROUND: Contrast-enhanced (ce) fluid-attenuated inversion recovery magnetic resonance imaging (FLAIR MRI) has recently been shown to identify leptomeningeal pathology in multiple sclerosis. OBJECTIVE: To demonstrate leptomeningeal enhancement on three-dimensional (3D) FLAIR in a case of Susac's syndrome. METHODS: Leptomeningeal enhancement was correlated with clinical activity over 20 months and compared to retinal fluorescein angiography. RESULTS: The size, number, and location of leptomeningeal enhancement varied over time and generally correlated with symptom severity. The appearance was remarkably similar to that of retinal vasculopathy. CONCLUSION: Ce 3D FLAIR may aid in diagnosis and understanding of pathophysiology in Susac's syndrome and may serve as a biomarker for disease activity.
BACKGROUND: Contrast-enhanced (ce) fluid-attenuated inversion recovery magnetic resonance imaging (FLAIR MRI) has recently been shown to identify leptomeningeal pathology in multiple sclerosis. OBJECTIVE: To demonstrate leptomeningeal enhancement on three-dimensional (3D) FLAIR in a case of Susac's syndrome. METHODS: Leptomeningeal enhancement was correlated with clinical activity over 20 months and compared to retinal fluorescein angiography. RESULTS: The size, number, and location of leptomeningeal enhancement varied over time and generally correlated with symptom severity. The appearance was remarkably similar to that of retinal vasculopathy. CONCLUSION: Ce 3D FLAIR may aid in diagnosis and understanding of pathophysiology in Susac's syndrome and may serve as a biomarker for disease activity.
Authors: S Coulette; A Lecler; E Saragoussi; K Zuber; J Savatovsky; R Deschamps; O Gout; C Sabben; J Aboab; A Affortit; F Charbonneau; M Obadia Journal: AJNR Am J Neuroradiol Date: 2019-06-27 Impact factor: 3.825