Association of Developmental Venous Anomalies with Demyelinating Lesions in Patients with Multiple Sclerosis

Abstract

We present 5 cases of demyelination in patients diagnosed with multiple sclerosis that are closely associated with a developmental venous anomaly. Although the presence of a central vein is a known phenomenon with multiple sclerosis plaques, demyelination occurring around developmental venous anomalies is an underreported phenomenon. Tumefactive demyelination can cause a diagnostic dilemma because of its overlapping imaging findings with central nervous system neoplasm. The relationship of a tumefactive plaque with a central vein can be diagnostically useful, and we suggest that if such a lesion is closely associated with a developmental venous anomaly, an inflammatory or demyelinating etiology should be a leading consideration.

Fig 1.

A and B, Axial contrast-enhanced and fat-saturated MR imaging show a right cerebellar DVA (A, arrow) surrounded by patchy parenchymal enhancement (B, arrow). C, Axial FLAIR MR imaging shows corresponding hyperintense lesion in the right cerebellum (arrow). D, Axial susceptibility-weighted imaging maximum intensity projection shows a large right cerebellar DVA (arrow). E, H&E staining 20× shows moderately hypercellular cerebellar white matter with infiltrating macrophages (circled). In addition, immunohistochemical staining demonstrated intact neurofilament and positive CD68 histiocytes, and Luxol fast blue stain showed myelin loss (not pictured).

Fig 2.

A and B, Sagittal contrast-enhanced T1 and FLAIR MR images show a large left frontal lobe DVA (A, arrow) surrounded by a lesion with discontinuous leading-edge enhancement (B, arrow), typical for tumefactive demyelination. C and D, Sagittal FLAIR MR images at the same anatomic location with a 15-month interval show the development of a demyelinating plaque adjacent to the occipital horn of the right lateral ventricle (arrows). E, Coronal T2 with fat-saturation MR imaging shows left optic nerve hyperintensity (arrow). The patient presented with optic neuritis 15 months before imaging in A and B. F, Axial contrast-enhanced T1 with fat saturation MR imaging shows left optic nerve enhancement (arrow) in the setting of optic neuritis.

Fig 3.

A, Sagittal FLAIR MR imaging shows multiple hyperintense callosal and pericallosal multiple sclerosis plaques (Dawson fingers; arrow) B, Axial T2 MR imaging shows a hyperintense left middle cerebellar peduncle lesion (midline arrow) associated with a flow void (central vein sign; left lateral arrow), which extends from the DVA better demonstrated in image C. C and D, Axial contrast-enhanced T1 MR images show an enhancing left middle cerebellar peduncle lesion (midline arrow) closely associated with a left cerebellar DVA (left lateral arrow). This enhancement was resolved on the follow-up scan at the same anatomic level 2 years later (D, arrow).

Fig 4.

A, Sagittal FLAIR MR imaging shows multiple hyperintense callosal and pericallosal multiple sclerosis plaques (consistent with Dawson fingers; arrow). B, Axial contrast-enhanced T1 and C, FLAIR MR images show a FLAIR hyperintense (C, arrow) and enhancing (B, arrow) lesion in the left frontal lobe. D and E, Coronal contrast-enhanced T1 MR images show an enhancing left frontal lobe lesion (D, arrow) closely associated with a DVA. This enhancement was resolved on the follow-up scan 9 months later (E, arrow).

Fig 5.

A, Axial contrast-enhanced T1 MR imaging shows a right parietal lobe DVA (arrow). B and C, Axial FLAIR MR images over an 8-month interval show development of right parietal lobe hyperintense plaques (B and C, arrows) oriented along the course of the DVA demonstrated in A.