J Clin Neurol.  2019 Oct;15(4):429-437. 10.3988/jcn.2019.15.4.429.

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

• Kim SW
• Kim TG
• Oh J
• Kim DY
• Choi YC
• Kim SM
• Shin HY
• Bang D

• KMID: 2467750
• DOI: http://doi.org/10.3988/jcn.2019.15.4.429

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.

Keyword

neuro-Behçet's disease; Behçet's disease; classification; treatment

MeSH Terms

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

Figure

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  Fig. 1 Flowchart of patient inclusion. CSF: cerebrospinal fluid, ISG: International Study Group, NBD: neuro-Behçet's disease.

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  Fig. 2 Classification of patients with NBD. Classification of 110 patients with NBD based on the clinical syndrome (A) and classification of 98 patients with parenchymal NBD based on the clinical course (B). NBD: neuro-Behçet's disease.

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  Fig. 3 Clinical manifestations in 98 patients with parenchymal neuro-Behçet's disease.

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