Neuro-Behçet’s Disease – Clinical Features, Diagnosis and Differential Diagnosis

Background: Behçet’s disease is a relatively uncommon, inflammatory disorder with characteristic mucocutaneous lesions and multisystem involvement, of unknown aetiology; presumably a vascular autoinflammatory syndrome that develops under combined environmental and genetic influences. As neuro-Behçet’s disease affects the central nervous system in about 10% of cases and in ways that can mimic other neuroinflammatory conditions, awareness of its manifestations, significance, and management is important for neurologists. In March 2017, a mini-symposium at the 11th Congress of Controversies in Neurology in Athens, Greece, was dedicated to specific aspects of Behçet’s and neuro-Behçet’s disease. These included an introduction to Behçet’s disease, pathogenesis and treatment, an overview of its neurological manifestations (neuro-Behçet’s disease) and the differential diagnosis from other neuroinflammatory conditions. Illustrative case reports were used. Objectives: To provide a brief overview of neuro-Behçet’s disease that is informative for clinical neurological practice and that follows the structure of the 2017 mini-symposium. Data sources: Relevant recent comprehensive reviews of the subject and relevant original articles and case reports were provided by each speaker at the mini-symposium. This article contains some of these sources and some additions where necessary to emphasise specific points. References are also provided for more comprehensive recent reviews. Limitations: The mini-symposium was an opportunity for providing a brief update and overview of neuro-Behçet’s disease and to exchange ideas and experience among neurologists. As such, it was found to be helpful, but also limited in scope. This resultant article refers to comprehensive reviews on the topic but is not in itself a comprehensive systematic review. Conclusions: Neuro-Bechet’s disease comprises largely two forms, parenchymal and a non-parenchymal. These manifestations seldom overlap in the same individual and may reflect different pathogenetic mechanisms. The principles of treatment largely follow the principles of treating Bechet’s disease in general, with the mainstay being corticosteroids for exacerbations and immunosuppressive treatments for prevention of exacerbations. One notable exception is cyclosporine, which is typically avoided in neuro-Bechet’s disease. Anti-tumour necrosis factor biologicals play an increasing role in treatment. Distinguishing neuro-Behçet’s disease from other neuroinflammatory conditions, such as multiple sclerosis, is essential for both management and prognostic reasons.