Neuro-Behçet's disease: epidemiology, clinical characteristics, and management

Behçet's disease (BD) is a multisystem relapsing inflammatory disorder of unknown cause. In neuro-BD (NBD), the CNS can be involved in one or both of two ways: first, and most commonly, through the development of an immune-mediated meningoencephalitis, which predominantly involves the brainstem, but can also involve the basal ganglia, thalamus, cortex and white matter, spinal cord, or cranial nerves; and second, as a consequence of thrombosis within the dural venous sinuses. Headache is a common symptom in BD and does not necessarily indicate CNS involvement. Peripheral nervous system involvement is rare. New treatment options have recently become available, which have led to an improvement in morbidity after meningoencephalitis. Most of the reported studies on NBD are retrospective. Collaborative prospective studies of the natural history of the disease, particularly the nature and treatment of progressive neurological disease, and evidence-based studies of treatment are needed.