Neuro-Behçet's Disease

Abstract

Behçet’s disease (BD) is an inflammatory multisystem disorder with clinical characteristics of uveitis, recurrent oral aphthae, vasculitis, and genital ulcerations. It may have vascular, musculoskeletal, gastrointestinal, mucocutaneous, ocular, central nervous system (neuro-Behçet’s disease), and many other systemic manifestations. Even though the etiopathogenesis of BD is still uncertain, virus infections, innate and adaptive immunity, genetic factors, and environmental contaminants have been mainly identified as responsible mechanisms. In this sense, the animal models of BD have been built up to enlighten the etiology of the disease. These models can be divided etiologically into four categories as infection-induced, genetic, immunological, and environmental models. The herpes simplex virus (HSV)-induced model is the most common and validated method among all other experimental designs of BD. The number of animal studies investigating neuro-Behçet disease (NBD) is very limited. Consequently, it is pertinent to say that further animal models regarding neurological component of BD should be developed to find new biomarkers for NBD and to advance treatment strategies.