Background Behçet's disease (BD) is a multisystem disease in which central nervous system involvement – neuro-Behçet's disease (NBD) – may strike young patients with devastating consequences. In this regard, early diagnosis and treatment is essential to prevent injury.
Objectives This study aimed to analyze NBD clinical features compared to non-neurological BD in order to distinguish disease patterns.
Methods A retrospective study was performed in 101 BD outpatients from a single tertiary center followed between 2011 and 2016. BD diagnosis was based on the 2014 International Criteria for Behçet's Disease. Demographic, clinical and imaging features of 28 NBD patients were compared to 73 BD patients without neurological involvement.
Results Earlier disease onset was found in NBD compared to BD (26.0±10.2 vs. 30.2±8.8 years, p=0.04). There were no differences between genders incidences, with a female predominance in both groups (64.3 vs. 72.6%, p>0.05). Over half of patients (53.6%) presented NBD as the first symptom and the mean time between diagnosis and NBD onset was 3.8±5.9 years. Uveitis was less frequent in NBD patients (25% vs. 47.9%, p=0.04), together with cutaneous disease (50% vs. 76.7%, p=0.01) and articular involvement (17.9% vs. 46.5%, p=0.01). Oral ulcers, genital ulcers, intestinal and vascular involvement frequencies were similar in both groups. Regarding NBD presentation, brainstem involvement was the most prevalent (67.9%), followed by central venous thrombosis (32.1%), aseptic meningitis (17.9%), stroke (3.6%) and peripheral neuropathy (3.6%). Most patients (82.1%) had a single neurological attack whereas relapsing disease was found in 18.5%.
Conclusions Our study found an earlier disease onset in NBD patients and a lower frequency of ocular, cutaneous and articular involvement. Moreover, several patients may unfold the disease as NBD, with lack of other manifestations. In addition, brainstem lesions occurred in most patients. Recognizing these disease patterns might support to expedite NBD diagnosis.
Disclosure of Interest None declared