Neuro-behçet's disease in South Korea: clinical characteristics and treatment response

Neuro-behçet's disease (NBD) is a rare complication of Behçet's disease (BD) but is still important due to its morbidity and mortality. In this study, we sought to identify the characteristics of NBD by examining the clinical characteristics, and whether there were differences in the clinical characteristics or the treatment between relapsed and non-relapsed groups.

We retrospectively reviewed the medical history of all patients diagnosed with BD at the Asan Medical Center in South Korea from January 1996 to May 2012. NBD was defined as neurological symptoms and compatible abnormalities identified by magnetic resononance imaging.

Among 624 BD patients, 22 were diagnosed with NBD. The common initial neurological symptoms were headache, gait disturbance and dysarthria. The common involved sites were the diencephalon, including internal capsule, the thalamus and basal ganglia, followed by the brainstem. An increased signal intensity on T2-weighted images was the most common finding. All patients had an improved disease state after steroid treatment, regardless of combined treatment with cyclophosphamide. However, eight patients experienced a disease recurrence at 7.3 ± 5.1 months after treatment. There were no significant differences in treatment patterns between the relapsed group and the non-relapsed group.

NBD may present with various neurologic manifestations with multifocal diencephalon and brainstem involvement. Most NBD patients had a good clinical outcome after steroid treatment, with or without a cytotoxic agent, but showed a pattern of relapse-remission. Therefore, careful long-term follow-up is needed in most cases of NBD.