Cerebral angio- and neuro-Behçet's syndrome: neuroradiological and pathological study of one case

Cerebral angio-Behçet's syndrome is extremely rare and pathological studies are scarce. We describe a 63-year-old man who developed left homonymous hemianopsia and hemiparesis 16 years after the onset of cardinal symptoms of Behçet's syndrome. CT, MRI and PET studies disclosed cerebral lesion with reduced neuronal metabolism in the right hemisphere, which was resolved by glucocorticoid therapy. Cerebral angiography showed no filling of the right Rolandic, anterior and posterior parietal and angular arteries. The postmortem study revealed: (a) occlusive panarteritis of some medium-sized pial branches of the right middle cerebral artery, considered as angio-Behçet's pathology, and small infarctions due to the vascular occlusion; (b) patchy or confluent demyelinated foci with perivascular lymphocytic infiltration in the bilateral brain basis, predominantly in the right retro- and sublenticular structures, being equivalent to neuro-Behçet's pathology. Cerebral angio- and neuro-Behçet's syndromes could have occurred and progressed concomitantly, which suggests a close relationship between the two subclassified processes.