Neurological Complications of Beh‡et's Syndrome

Beh double dagger et's syndrome is an autoinflammatory disease of uncertain cause characterized by mucous membrane ulceration, arthritis and inflammation within most tissues. Its prevalence differs throughout different populations, but neurological complications arise in 10 % of all those affected. The majority develop inflammation within the central nervous system which may remit but may also progressively worsen without treatment. This article reviews the epidemiology, clinical characteristics, natural history and management of the disorders, and emphasizes recent considerable advances in our understanding of the various treatments which may be beneficial across the spectrum of disease types.