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Clinical and Radiographic Characteristics of Neuro-Behçet's Disease in South Korea

 Seung Woo Kim  ;  Tae-Gyun Kim  ;  Jongwook Oh  ;  Do-Young Kim  ;  Young-Chul Choi  ;  Seung Min Kim  ;  Ha Young Shin  ;  Dongsik Bang 
 JOURNAL OF CLINICAL NEUROLOGY, Vol.15(4) : 429-437, 2019 
Journal Title
Issue Date
Behçet's disease ; classification ; neuro-Behçet's disease ; treatment
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.
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1. College of Medicine (의과대학) > Dept. of Dermatology (피부과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Neurology (신경과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Do Young(김도영) ORCID logo https://orcid.org/0000-0002-0194-9854
Kim, Seung Min(김승민) ORCID logo https://orcid.org/0000-0002-4384-9640
Kim, Seung Woo(김승우) ORCID logo https://orcid.org/0000-0002-5621-0811
Shin, Ha Young(신하영) ORCID logo https://orcid.org/0000-0002-4408-8265
Oh, Jongwook(오종욱)
Choi, Young Chul(최영철) ORCID logo https://orcid.org/0000-0001-5525-6861
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