Symptomatic Developmental Venous Anomaly with an Increased β2-microglobulin Level in Cerebrospinal Fluid: A Case Report

Background: Gadolinium-enhanced magnetic resonance imaging (MRI) can be used to observe the progression of cerebral infarction, which sometimes mimics malignant brain tumors. While the β2-microglobulin (β2MG) level in blood plasma or cerebrospinal fluid (CSF) is useful for the diagnosis of malignant tumors or degenerative diseases, these results may create confusion regarding a definitive diagnosis, because it is not a specific marker. We present a rare case of symptomatic developmental venous anomaly (DVA), accompanied by transient, irregular, enhanced cerebral lesions and elevated β2MG in the CSF.
Case Description: A 56-year-old woman developed dysarthria and underwent MRI, which revealed a right frontal hyperintense area around a previous lesion on diffusion-weighted imaging (DWI). She was treated based on the tentative diagnosis of an ischemic cerebrovascular event, and symptoms subsided in 3 days. MRI on day 7 revealed an enlargement of the hyperintense area on DWI. Post-gadolinium MRI showed multiple, enhanced patchy areas in the right frontal lobe and an abnormally large vein connected to dilated medullary venules, indicating DVA. Magnetic resonance angiography showed no stenosis or arterial occlusion. The β2MG level in the CSF was elevated at 2,061 μg/l, and a differential diagnosis from malignant tumor was required. However, MRI on day 23 revealed total disappearance of the enhanced lesions and a decrease in the high intensity area on DWI. Considering the clinical course, the DVA was symptomatic because of the perfusion disturbance.
Conclusion: Careful evaluation is necessary when considering the associated pathologies and potential complications of DVA if detected near a gadolinium-enhanced lesion.