Diagnostic Relevance of the Medullary Vein Sign in Neurosarcoidosis
Samiksha Srivastava1, Kanika Sharma2, Joe Joseph3, Parissa Feizi3, Shitiz Sriwastava3
1Internal Medicine, Sinai Grace Hospital-DMC, 2Neurology, West Virginia Clinical & Translational Science Institute, Morgantown, WV, 3Neurology, Department of Neurology, Rockefeller Neuroscience Institute, West Virginia University
Objective:

To evaluate the diagnostic potential of the medullary vein sign as an imaging marker in neurosarcoidosis.

Background:

Neurosarcoidosis is a rare manifestation of sarcoidosis that can be inferred through the clinical presentation, cerebral spinal fluid (CSF) analysis, and magnetic resonance imaging (MRI) scans. However, its accurate diagnosis remains a significant challenge due to the variability in its diagnostic criteria and the overlapping clinical and imaging features with other conditions. One under-researched finding in these patients is the engorgement of the medullary vein on susceptibility-weighted imaging (SWI), termed as the 'medullary vein sign'; postulated to be a specific sign for neurosarcoidosis, its reliability has not yet been fully elucidated.

Design/Methods:

A single-center retrospective study was performed between March 31st, 2022, and August 1st, 2022. Two neuroradiologists independently examined MRI images using SWI/gradient echo sequences for medullary vein engorgement. A total of 32 patients were reviewed, and the diagnosis was based on imaging, biopsy, and CSF analysis. Finally, the sensitivity and specificity of the medullary vein sign were calculated.

Results:
Out of 32 patients, seven were diagnosed with definitive neurosarcoidosis; the remaining were classified as possible (n=16), probable (n=5), and other (n=4) causes of infectious meningoencephalitis, including two cases of autoimmune encephalitis. Among these, the medullary vein sign was detected in seven patients: five with confirmed, and two with possible neurosarcoidosis. Overall, the sensitivity was 71.4%, and the specificity was 92.3%. CSF was largely inconclusive, and MRI was not the major diagnostic factor in most cases.
Conclusions:

Our findings confirm the high specificity of the medullary vein sign for neurosarcoidosis and suggest that SWI is an effective tool for early detection, especially compared to conventional MRI and CSF analysis. Incorporating this in the current diagnostic criteria may result in timely intervention, and we encourage radiologists to look for this marker in patients with suspected neurosarcoidosis.

10.1212/WNL.0000000000202032