Diagnosing neuro-Behçet’s disease

Behçet’s disease is a rare systemic vasculitis characterized by uveitis, recurrent oral and genital ulcers, firstly described by the Turkish dermatologist Hulusi Behçet. The etiology is unknown, although autoimmune mechanisms are described. There is no specific test for the diagnosis of Behçet’s disease. The International Criteria for Behçet’s Disease (ICBD) proposed a new set of diagnostic criteria including oral and genital aphthosis, skin, ocular and vascular manifestations, CNS involvement and positive pathergy test. The neurologic involvement could be classified in parenchymal neuro-Behçet’s and non-parenchymal neuro-Behçet’s disease. We report a case of a woman with a very impressive personal history. Over time, she had many specific neurological complications compatible with neuro-Behçet’s disease (NBD). The other general symptoms also suggested Behçet’s disease, according to ICBD.