Neurobehçet disease: clinical and demographic characteristics

Abstract

Neurobehçet disease (NBD) is a rare complication of Behçet disease (BD) but with important burdens of morbidity and mortality. Little is known about this complication because there are no validated diagnostic criteria, and all the studies have small number of patients. The prevalence reported normally ranges between 5% and 15% and it is more frequent amongst men between 20 and 40 years old. The typical presentations include focal parenchymal lesions, vascular thrombosis, arterial vasculitis, and aseptic meningo-encephalitis. We retrospectively studied medical histories of all patients admitted to the hospital and discharged from it with diagnosis of BD from January 1996 to September 2009. NBD was defined as having neurological and/or psychiatric symptoms with compatible abnormalities in MRI and/or cerebrospinal fluid and without another possible explanation for their symptoms. Behcet disease was diagnosed in 25 patients and seven from these patients fulfilled our criteria of Neurobehcet disease (28%). Patients with NBD were significantly younger at the onset of their symptoms and had a significantly longer evolution until diagnosis and treatment compared to patients with non-Neuobehçet disease. Six presented a relapsing-remitting pattern, with a good outcome with corticosteroids. As reported in previous studies, progressive course was less frequent, with only one case, and had a more aggressive disease. Brainstem involvement bears a poorer prognosis because it is linked with a progressive evolution. In our series, NBD complication was not that infrequent. It is very important to be highly suspicious of this possibility to start early a correct treatment.