Kim Seung Woo, Kim Tae Gyun, Oh Jongwook, Kim Do Young, Choi Young Chul, Kim Seung Min, Shin Ha Young, Bang Dongsik
Department of Neurology, Yonsei University College of Medicine, Seoul, Korea.
Department of Dermatology, Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
J Clin Neurol. 2019 Oct;15(4):429-437. doi: 10.3988/jcn.2019.15.4.429.
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.
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.
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.
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.
白塞病的神经系统受累[神经白塞病（NBD）]并不常见，但因其可导致严重残疾，故而值得深入研究。然而，由于存在地区差异以及缺乏采用公认诊断标准的研究，了解NBD的临床特征存在困难。因此，我们依据最近的国际共识建议，分析了NBD患者的临床特征。
我们对2000年至2017年间白塞病（BD）患者的电子数据库进行了回顾性检索，并查阅了他们的病历。依据最近的国际共识建议，纳入了确诊或疑似NBD的患者。
在9817例诊断代码为BD的患者中，1682例（17.1%）前往神经科就诊，110例（1.1%）被归类为NBD。98例表现为实质性NBD，12例表现为非实质性NBD。他们NBD发病时的年龄为37.6±10.6岁，男女比例为1.24:1。在98例实质性NBD患者中，脑干综合征（43.9%）最为常见，其次是多灶性（32.7%）和脊髓（12.2%）综合征。72.4%表现为急性NBD，27.6%表现为疾病进展过程。常见表现包括锥体束征（52.0%）、头痛（45.9%）、构音障碍（42.9%）和发热（31.6%）。脑部MRI的常见表现是上脑干病变延伸至丘脑和基底节。
约1%疑似BD的患者表现为NBD。神经科医生必须了解NBD的临床特征，以便对这些患者进行鉴别诊断和管理。
