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

Kim, Seung-Woo; Kim, Tae Kyun; Oh, Jongwook; Kim, Do Young; Choi, Young Chul; Shin, Ha Young; Bang, Dongsik

doi:10.3988/jcn.2019.15.4.429

Cited by 8 publications

(1 citation statement)

References 41 publications

(83 reference statements)

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“…Vascular BD lesions can involve both arteries and veins, but deep vein thrombosis of the lower extremities is the most common presentation [ 17 ]. One of the largest cohorts on neuro-BD in Korea showed brainstem manifestations (43.9%) to be the most common, followed by multifocal (32.7%) and spinal cord (12.2%) manifestations [ 18 , 19 ]. About 27% of patients in this analysis exhibited a progressive disease course, thus highlighting the necessity of careful long-term follow-up in BD patients with neurological symptoms.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Epidemiologic and Etiological Features of Korean Patients With Behçet's Disease

Choi

Kim

2021

J Rheum Dis

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Behçet's disease (BD) is a multisystem disease in which environmental factors provoke an adverse immune response in patients with genetic susceptibility towards BD, subsequently leading to a cascade of dysregulated inflammation throughout the body. It is particularly prevalent in regions spanning the ancient Silk Road, including Korea, where the first known case of BD was reported in 1961. We summarize the history, epidemiology, and clinical presentation of BD in Korea, highlighting the clinical tendencies that are particularly seen in the Korean BD population as compared to European populations. Analysis of epidemiologic trends over the past three decades in Korea shows a decreasing prevalence of complete BD and a higher prevalence of intestinal BD. We also discuss the ever-evolving understanding of the pathogenesis of BD, noting the complex interplay among genetics, environment, and immunology. The HLA-B51 allele is the most significant known genetic risk factor in developing BD. We also discuss more recently studied associations between BD and immune factors such as IL-10, IL-23R-IL-12RB2, IL-1A-IL-1B, CCR1, ERAP1, and the GIMAP cluster, the last of which has been found to have an association with BD specifically in Korea. Environmental factors such as pollution and microbials are often the inciting event in developing BD, as they trigger an imbalanced immune response in genetically susceptible individuals, one that has been often found to exhibit an aberrant Th1/Th17 response. There would be value to further studying the pathogenesis and clinical characteristics of Korean BD.

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Section: Clinical Manifestationsmentioning

confidence: 99%

Epidemiologic and Etiological Features of Korean Patients With Behçet's Disease

Choi

Kim

2021

J Rheum Dis

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Prognostic factors for relapse and poor outcome in neuro-Behçet’s syndrome: results from a clinical long-term follow-up of a single centre

et al. 2021

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Diffusion tensor imaging in Behcet’s disease with and without neurological involvement patients: evaluation of microstructural white matter abnormality with a tract-based spatial statistical analysis

Aslan,

Genç,

Bolat

et al. 2024

British Journal of Radiology

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Objective This study aims to assess the microstructural abnormalities in white matter (WM) among Behcet's Disease (BD) patients, both with and without neurological involvement, utilizing tract-based spatial statistics (TBSS) to elucidate the underlying causes of WM microstructural changes. Methods This prospective study comprised 43 BD patients without neurological involvement, 15 Neuro-Behcet's Disease (NBD) patients with normal conventional MRI, and 54 healthy controls matched for age and sex. TBSS was applied in this diffusion tensor imaging study to conduct a whole-brain voxel-wise analysis of fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) of WM. Results Compared to the control group, BD patients exhibited decreased FA and increased MD and RD in nearly all WM tracts, along with increased AD in the left corticospinal tract (CST), left inferior longitudinal fasciculus (ILF), and left superior longitudinal fasciculus (SLF). NBD patients also showed a widespread decrease in FA and increased MD and RD, similar to BD patients without neurological involvement. Additionally, NBD patients had increased AD in the left CST, left ILF, left SLF, left inferior fronto-occipital fasciculus (IFOF), and right CST. Compared to BD patients without neurological involvement, NBD patients exhibited a greater reduction in FA and an increase in MD and RD in WM tracts, with no significant differences in AD. Conclusion These results suggest that the main mechanism of microstructural changes in the WM of BD patients may be related to impaired fiber integrity, demyelination, decreased myelin sheath integrity. Advances in knowledge This study demonstrated BD patients without neurological involvement and NBD patients a decrease in FA and an increase in MD and RD were observed in larger areas of major WM tracts, while an increase in AD values was observed in fewer tracts. Our findings may be useful in understanding the pathophysiology underlying subclinical parenchymal involvement and neurological dysfunction in BD patients and in the management of BD patients.

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

Cited by 8 publications

References 41 publications

Epidemiologic and Etiological Features of Korean Patients With Behçet's Disease

Epidemiologic and Etiological Features of Korean Patients With Behçet's Disease

Prognostic factors for relapse and poor outcome in neuro-Behçet’s syndrome: results from a clinical long-term follow-up of a single centre

Diffusion tensor imaging in Behcet’s disease with and without neurological involvement patients: evaluation of microstructural white matter abnormality with a tract-based spatial statistical analysis

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