11 - BEHÇET’S DISEASE

Summary

Behçet's disease (BD) is a multisystemic inflammatory disorder of unknown etiology, and neurologic involvement is one of the major clinical features. When the clinicopathological and neuroradiological findings are combined, two different patterns of central nervous system (CNS) involvement in BD can be established: parenchymal and neurovascular. Neurologic involvement is one of the most devastating manifestations of BD. This involvement may occur primarily within the nervous parenchyma (n-BD) or secondarily in the cerebral vascular system (vasculo[angio]-BD). Thrombosis of cerebral large veins and sinuses is the most common feature of vasculo-BD, although thrombosis of the vena cava and portal vein may also occur in one third of these patients. BD is usually included among the systemic vasculitides but documented cerebral arteritis is extremely rare and even a debatable mechanism for CNS involvement. Cerebrovascular complications of BD are rarer than parenchymal involvement of the CNS and aseptic meningitis.