Association of a giant developmental venous anomaly and acute disseminated encephalomyelitis: A case report and magnetic resonance perfusion study

Abstract

We describe the first reported association between acute disseminated encephalomyelitis (ADEM) and giant developmental venous anomaly (DVA) in the context of myelin oligodendrocyte associated glycoprotein (MOG) associated disorder (MOGAD). Patient was a young woman presenting with headache, bradypsychia and tetrapyramidal syndrome. Imaging showed disseminated tumefactive inflammatory lesions in the brain and spinal cord, with a massive right frontal lobe lesion centred around a giant DVA. Demyelinating inflammatory lesions are known to occur in a perivenular pattern, and the association between some inflammatory diseases such as multiple sclerosis (MS) and DVA has already been described. Developmental venous anomalies are variant of the normal venous drainage of the brain, responsible of a local alteration of the venular network, and micro-perfusion anomalies as well as possible increased of blood-brain barrier permeability. As such, they might be responsible for a favourable environment for pathogenic auto-antibodies penetrance in such region, potentializing the inflammatory lesion size. Perfusion imaging showed a significant increase in regional blood volume and blood transit time in the DVA and the surrounding brain tissue, which regressed in the follow-up imaging studies after the acute stage. This case illustrates the potential role of DVA in the setting of demyelinating diseases, and its consequences on the local micro-perfusion of the brain, evolving between the acute and chronic phase of the illness.

Keywords: ADEM; MOGAD; demyelination; developmental venous anomaly; perfusion imaging.

Figure 1.

Left panel: emergent non-contrast-enhanced computed tomography (CT) brain scan, showing a massive hypodense lesion in the right frontal lobe. Right panel: contrast-enhanced CT, showing a giant DVA in the right frontal lobe.

Figure 2.

(A) FLAIR ponderated sequence, showing a massive tumefactive inflammatory lesion in the right frontal lobe. (B) Gradient-echo T1 ponderated sequence with gadolinium, showing the right frontal lobe DVA. (C) Frontal maximum intensity projection (MIP) showing the same DVA. (D) Short TI Inversion recovery sagittal sequence, showing an hyper-T2 lesion in the medullar cord at C3–C4 level. (E) rCBV map, showing a massive increase in relative blood volume in the area of the DVA and inflammatory lesion. (F) FDG-PET scan showing a decrease in brain glucose intake in the right frontal lobe. (G) SWI MIP sequence, showing a relatively low vascular hyposignal in the area of the DVA. (H) FLAIR ponderated sequence, showing multiples other ADEM lesions in hypersignal trough the brain.

Figure 3.

Top row: acute magnetic resonance examination. Bottom row: 3 months follow-up examination. (A and E) FLAIR ponderated sequence, showing the regression of oedema between the acute (A) and follow-up (E) examinations. (B and F) SWI MIP sequence, showing the relative increase in hyposignal the DVA venular network and main collector between acute (B) and follow-up (F) exams. (C and G) rCBV maps, showing a relative decrease of regional blood volume in the DVA between acute and follow-up exam. (D and H) tMIP maps, showing relative decrease in the slowness of the regional blood drainage in the DVA area between acute and follow-up exams.