Background and Purpose Neurological involvement in Behçet’s disease [neuro-Behçet’s disease (NBD)] is uncommon, but it is worth investigating since it can cause substantial disability. However, difficulties exist in understanding the clinical features of NBD due to regional variations and the lack of studies utilizing well-established diagnostic criteria. We therefore analyzed the clinical features of patients with NBD based on the recent international consensus recommendation. Methods We retrospectively searched electronic databases for patients with Behçet’s disease (BD) between 2000 and 2017, and reviewed their medical records. Based on the recent international consensus recommendation, patients with definite or probable NBD were included. Results Of 9,817 patients with the diagnosis code for BD, 1,682 (17.1%) visited the neurology clinic and 110 (1.1%) were classified as NBD. Ninety-eight patients exhibited parenchymal NBD and 12 exhibited nonparenchymal NBD. Their age at the onset of NBD was 37.6±10.6 years and the male-to-female ratio was 1.24:1. Brainstem syndrome (43.9%) was the most common condition in the 98 patients with parenchymal NBD, followed by multifocal (32.7%) and spinal cord (12.2%) syndromes. 72.4% exhibited acute NBD and 27.6% exhibited a progressive disease course. Frequent manifestations included pyramidal signs (52.0%), headache (45.9%), dysarthria (42.9%), and fever (31.6%). A frequent pattern in brain MRI was an upper brainstem lesion extending to the thalamus and basal ganglia. Conclusions Approximately 1% of the patients with suspected BD exhibited NBD. Neurologists must understand the clinical characteristics of NBD in order to perform the differential diagnosis and management of these patients.
Bibliographical noteFunding Information:
This work was supported by the National Key R&D Program of China (2016YFC1000207) and the National Natural Science Foundation of China (grant 81630085), and key projects of visiting scholars at home and abroad in 2016 (grant gxfxZD2016158), Six Talent Peaks Project of Jiangsu Province (grant JY-052), and State Key Laboratory of Reproductive Medicine of Nanjing Medical University (SKLRM; grant KY116NJMUKL16004). We declare that we have no conflicts of interest.
© 2019 Korean Neurological Association.
All Science Journal Classification (ASJC) codes
- Clinical Neurology