Siva A, Kantarci O H, Saip S, Altintas A, Hamuryudan V, Islak C, Koçer N, Yazici H
Department of Neurology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey.
J Neurol. 2001 Feb;248(2):95-103. doi: 10.1007/s004150170242.
This study was conducted to describe clinical and prognostic aspects of neurological involvement in Behçet's disease (BD). Patients referred for neurological evaluation fulfilled the criteria of the International Study Group for Behçet's Disease. We analyzed disability and survival by the Kaplan-Meier method, using Kurtzke's Extended Disability Status Scale (modified for BD) and the prognostic effect of demographic and clinical factors by Cox regression analysis. We studied 164 patients; of the 107 diagnostic neuroimaging studies: 72.1% showed parenchymal involvement, 11.7% venous sinus thrombosis (VST) and the others were normal. CSF studies were performed in 47 patients; all with inflammatory CSF findings (n = 18) had parenchymal involvement. An isolated increase in pressure was compatible with either VST or normal imaging. The final diagnoses were VST (12.2%), neuro-Behçet syndrome (NBS) (75.6%), isolated optic neuritis (0.6%), psycho-Behçet syndrome (0.6%), and indefinite (11%). VST and NBS were never diagnosed together. Ten years from onset of BD 45.1% (all NBS) reached a disability level of EDSS 6 or higher, and 95.7 +/- 2.1% of the patients were still alive. Having accompanying cerebellar symptoms at onset or a progressive course is unfavorable. Onset with headache or a diagnosis of VST is favorable. Two major neurological diagnoses in BD are NBS and VST. These are distinct in clinical, radiological, and prognostic aspects, hence suggesting a difference in pathogenesis.
本研究旨在描述白塞病（BD）神经受累的临床及预后情况。因神经方面问题接受评估的患者符合白塞病国际研究组的标准。我们采用Kaplan-Meier方法分析残疾情况和生存率，使用库尔茨克扩展残疾状态量表（针对BD进行了修改），并通过Cox回归分析人口统计学和临床因素的预后影响。我们研究了164例患者；在107项诊断性神经影像学研究中：72.1%显示实质受累，11.7%显示静脉窦血栓形成（VST），其他结果正常。对47例患者进行了脑脊液检查；所有脑脊液有炎症表现的患者（n = 18）均有实质受累。单纯压力升高与VST或影像学正常均相符。最终诊断为VST（12.2%）、神经白塞综合征（NBS）（75.6%）、孤立性视神经炎（0.6%）、精神白塞综合征（0.6%）以及不确定（11%）。VST和NBS从未同时被诊断。从BD发病起10年，45.1%（均为NBS）达到扩展残疾状态量表（EDSS）评分为6或更高的残疾水平，95.7±2.1%的患者仍存活。发病时伴有小脑症状或病程呈进行性是不利的。以头痛起病或诊断为VST是有利的。BD的两种主要神经诊断是NBS和VST。它们在临床、放射学和预后方面各不相同，因此提示发病机制存在差异。
