Contributions
Abstract: P1063
Type: Poster
Abstract Category: Pathology and pathogenesis of MS - Imaging
Introduction: Susac"s syndrome (SS) is an autoimmune, microendotheliopathy affecting the retina, cochlea, and cerebrum. It is characterized clinically by branch retinal artery occlusion, encephalopathy, and hearing loss. Brain MRI shows characteristic white matter lesions with predilection for the central corpus callosum. Multiple sclerosis is a chronic demyelinating disease for which SS is a common differential diagnosis. Biomarkers to differentiate these two conditions are needed, and central vein sign identification has been proposed as a potentially useful non-invasive method for discrimination.
Methods: After IRB approval, 3 patients with relapsing remitting multiple sclerosis and 3 patients with SS were enrolled into a cross-sectional imaging study. Ultra high field MRI at 7T was acquired for all patients. 7T MRI sequences included 3D MP2RAGE (0.75 mm3 isotropic voxel, flip angle = 4° and 5°, TI1/TI2 = 700/2600 ms), 3D FLAIR (1mm3 isotropic voxel), 2D GRE T2* (0.38 x 0.39 x 1.5 mm3, flip angle = 20°), 3D SWI (0.49 x 0.49 x 0.8 mm3, flip angle = 20°). White matter lesions where identified on T2 FLAIR images measuring greater than 3 mm and then examined on GRE and SWI sequences for identification of a central vein. A determination of central vein presence was made when a central area of GRE/SWI hypo-intensity, as a thin line (when imaged across the long axis) or dot (when imaged across the short axis) were seen. Proportions were derived for presence/absence of a central vein in each group. Fisher"s exact test was used to compare the proportion of central veins in the multiple sclerosis and SS groups.
Results: A total of 105 lesions were identified in MS subjects. Central veins were identified in 85 (81%) of MS lesions. No central vein was identified in 18 lesions (17.1%), and 2 lesions (1.9%) were excluded due to uncertainty regarding central vein presence. A total of 22 lesions were detected in the SS patients, with only 1 lesion showing a central vein (4.5%). The proportion of central vein lesions was statistically higher in multiple sclerosis as compared to SS (Fisher"s exact test p < 0.0001). One SS lesion demonstrated a peri-lesional rim of GRE hypo-intensity.
Conclusion: The central vein sign at 7T is a viable measure to differentiate MS and SS. As described in prior studies, the majority of MS lesions demonstrate a central vein. The presence of a rim of GRE/SWI hypo-intensity should be explored further as a diagnostic marker of SS.
Disclosure: Daniel Ontaneda reports grant support form NIH, Genzyme, Novartis and consulting fees from Genentech, Novartis, and Biogen Idec
Se-Hong Oh has nothing to disclose
Robert Rennebohn has nothing to disclose
Mark Lowe has nothing to disclose
Steven Jones has nothing to disclose
Devon Conway reports consulting fees from Arena Therapeutics
Abstract: P1063
Type: Poster
Abstract Category: Pathology and pathogenesis of MS - Imaging
Introduction: Susac"s syndrome (SS) is an autoimmune, microendotheliopathy affecting the retina, cochlea, and cerebrum. It is characterized clinically by branch retinal artery occlusion, encephalopathy, and hearing loss. Brain MRI shows characteristic white matter lesions with predilection for the central corpus callosum. Multiple sclerosis is a chronic demyelinating disease for which SS is a common differential diagnosis. Biomarkers to differentiate these two conditions are needed, and central vein sign identification has been proposed as a potentially useful non-invasive method for discrimination.
Methods: After IRB approval, 3 patients with relapsing remitting multiple sclerosis and 3 patients with SS were enrolled into a cross-sectional imaging study. Ultra high field MRI at 7T was acquired for all patients. 7T MRI sequences included 3D MP2RAGE (0.75 mm3 isotropic voxel, flip angle = 4° and 5°, TI1/TI2 = 700/2600 ms), 3D FLAIR (1mm3 isotropic voxel), 2D GRE T2* (0.38 x 0.39 x 1.5 mm3, flip angle = 20°), 3D SWI (0.49 x 0.49 x 0.8 mm3, flip angle = 20°). White matter lesions where identified on T2 FLAIR images measuring greater than 3 mm and then examined on GRE and SWI sequences for identification of a central vein. A determination of central vein presence was made when a central area of GRE/SWI hypo-intensity, as a thin line (when imaged across the long axis) or dot (when imaged across the short axis) were seen. Proportions were derived for presence/absence of a central vein in each group. Fisher"s exact test was used to compare the proportion of central veins in the multiple sclerosis and SS groups.
Results: A total of 105 lesions were identified in MS subjects. Central veins were identified in 85 (81%) of MS lesions. No central vein was identified in 18 lesions (17.1%), and 2 lesions (1.9%) were excluded due to uncertainty regarding central vein presence. A total of 22 lesions were detected in the SS patients, with only 1 lesion showing a central vein (4.5%). The proportion of central vein lesions was statistically higher in multiple sclerosis as compared to SS (Fisher"s exact test p < 0.0001). One SS lesion demonstrated a peri-lesional rim of GRE hypo-intensity.
Conclusion: The central vein sign at 7T is a viable measure to differentiate MS and SS. As described in prior studies, the majority of MS lesions demonstrate a central vein. The presence of a rim of GRE/SWI hypo-intensity should be explored further as a diagnostic marker of SS.
Disclosure: Daniel Ontaneda reports grant support form NIH, Genzyme, Novartis and consulting fees from Genentech, Novartis, and Biogen Idec
Se-Hong Oh has nothing to disclose
Robert Rennebohn has nothing to disclose
Mark Lowe has nothing to disclose
Steven Jones has nothing to disclose
Devon Conway reports consulting fees from Arena Therapeutics