Behçet’s Disease and Neuro-Behçet’s Syndrome

Abstract

Behçet’s disease is an idiopathic chronic relapsing multisystem vascular-inflammatory disease of unknown origin, which usually presents with orogenital ulceration and uveitis and is identified as the triple-symptom complex. Primary neurological involvement in Behçet’s disease is known as neuro-Behçet’s syndrome (NBS). Clinical findings and neuroimaging demonstrate that there are two major forms of NBS: a central nervous system inflammatory parenchymal disease, and a less common nonparenchymal form that involves the large extraparenchymal vascular structures, mainly the venous dural sinuses. Cranial magnetic resonance imaging (MRI) typically reveals brainstem lesions with parenchymal involvement and an occluded dural sinus may be seen in the extraparenchymal type. Cerebrospinal fluid studies typically indicate inflammatory changes in the parenchyma and increased pressure with extraparenchymal involvement. Drugs used for the preventive treatment of NBS include azathioprine, cyclophosphamide, and anti-TNF agents.