Clinical and Radiographic Characteristics of Neuro-Behçet's Disease in South Korea
10.3988/jcn.2019.15.4.429
- Author:
Seung Woo KIM
1
;
Tae Gyun KIM
;
Jongwook OH
;
Do Young KIM
;
Young Chul CHOI
;
Seung Min KIM
;
Ha Young SHIN
;
Dongsik BANG
Author Information
1. Department of Neurology, Yonsei University College of Medicine, Seoul, Korea. hayshin@yuhs.ac
- Publication Type:Original Article
- Keywords:
neuro-Behçet's disease;
Behçet's disease;
classification;
treatment
- MeSH:
Basal Ganglia;
Brain;
Brain Stem;
Classification;
Consensus;
Diagnosis;
Diagnosis, Differential;
Dysarthria;
Fever;
Headache;
Humans;
Korea;
Magnetic Resonance Imaging;
Medical Records;
Neurology;
Retrospective Studies;
Spinal Cord;
Thalamus
- From:Journal of Clinical Neurology
2019;15(4):429-437
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.
