背景:患有Behçet病(BD)的患者很少表现为类似于多发性硬化(MS)的脑白质病变。由于实质神经BD(pNBD)和MS之间的诊断困难,这可能导致误诊。本研究旨在通过关注临床和实验室特征,阐明BD和MS(BDMS)合并症患者与仅pNBD和MS患者的区别特征。我们还旨在通过将BD+MS患者与pNBD和MS患者进行比较来确定BD+MS患者的独特特征。
方法:本研究的方法涉及对伊斯坦布尔医学院神经内科患者记录的回顾性分析,伊斯坦布尔大学。研究人群包括诊断为pNBD的患者,MS,BD和MS(BD+MS)共病。我们评估了临床,放射学,和实验室数据,包括疾病发作,年复发率,扩展的残疾状况量表(EDSS)进展,和脑脊液检查。在pNBD之间检查了几个参数,MS,和BD+MS患者组,以发现亚组之间的异同。
结果:我们的研究包括1,764例患者:172例pNBD,1,574与MS,和18与BD+MS。在BD+MS中,女性占主导地位(72%,p<0.001)和MS(69%,与pNBD(30%)相比,p<0.001)组。BD+MS出现神经系统症状的中位年龄为35.5(IQR:16.8)岁,pNBD为34.6(13.6)年,MS为27.6(13.3)年(BD+MSvs.MS;p=0.3,pNBD与MS,p=0.7)。此外,攻击的数量明显不同,BD+MS患者的发作中位数为3.5(2.0),而MS患者为3.0(3.0),pNBD患者仅为1.0(1.0),提示与pNBD相比,MS和BD+MS组的病程更活跃(p<0.001)。BD+MS的中位年复发率为0.3(0.2),低于MS(p=0.048)中0.4(0.4)的比率,相当于pNBD中0.2(0.3)的比率(p=0.2)。第一次复发的时间与BD+MS和MS相似,但明显短于pNBD患者(p<0.0001)。脑脊液(CSF)分析显示BDMS和MS患者的中性粒细胞和淋巴细胞计数没有显着差异,但pNBD患者的水平升高(p<0.05)。所有组的CSF蛋白水平是一致的(p=0.1和p=0.7)。在所有BD+MS患者中均检测到寡克隆条带,在大多数MS患者(83.6%)中,和一小部分pNBD患者(19.7%),显示BD+MS和pNBD组之间的显著区别(p<0.001)。
结论:我们的研究强调了在诊断和治疗表现出症状性MS样MRI病变的BD患者中需要一种怀疑的方法。我们的研究结果表明,BD+MS是一种独特的临床实体,保证具体的诊断和治疗方法。我们的发现强调,符合MS诊断标准的MS样病变的BD患者应作为MS和BD合并症而不是pNBD患者进行管理。
BACKGROUND: Patients with Behçet\'s disease (BD) may rarely manifest with cerebral white matter lesions resembling multiple sclerosis (MS). This may result in misdiagnosis due to diagnostic difficulties between parenchymal neuro-BD (pNBD) and MS. This study aims to elucidate the distinguishing features of patients with comorbid BD and MS (BD+MS) in comparison to those with pNBD and MS alone by focusing on clinical and laboratory features. We also aimed to identify the distinctive characteristics of BD+MS patients by comparing them to patients with pNBD and MS.
METHODS: The methodology of this study involved a retrospective analysis of patient records followed in the Department of Neurology at the Istanbul Faculty of Medicine, Istanbul University. The study population included patients diagnosed with pNBD, MS, and a comorbid condition of BD and MS (BD+MS). We assessed clinical, radiological, and laboratory data, including disease onset, annual relapse rates, Expanded Disability Status Scale (EDSS) progression, and cerebrospinal fluid examination. Several parameters were examined between the pNBD, MS, and BD+MS patient groups to find similarities and differences between subgroups.
RESULTS: Our study included 1,764 patients: 172 with pNBD, 1,574 with MS, and 18 with BD+MS. A predominance of females was noted in the BD+MS (72%, p < 0.001) and MS (69 %, p < 0.001) groups compared to pNBD (30 %). The median age at the onset of neurological symptoms was 35.5 (IQR: 16.8) years for BD+MS, 34.6 (13.6) years for pNBD, and 27.6 (13.3) years for MS (BD+MS vs. MS; p = 0.3, pNBD vs. MS, p = 0.7). Additionally, the number of attacks was notably different, with BD+MS patients experiencing a median of 3.5 (2.0) attacks compared to 3.0 (3.0) for MS patients and only 1.0 (1.0) for pNBD patients, suggesting a more active disease course in the MS and BD+MS groups compared to pNBD (p < 0.001). The median annualized relapse rate for BD+MS was 0.3 (0.2), which was lower than the rate of 0.4 (0.4) in MS (p = 0.048) and equivalent to the rate of 0.2 (0.3) in pNBD (p = 0.2). The time to the first relapse was similar to those with BD+MS and MS, but considerably shorter than in individuals with pNBD (p < 0.0001). The cerebrospinal fluid (CSF) analysis showed no significant differences in neutrophil and lymphocyte counts between BD+MS and MS patients but elevated levels in pNBD patients (p < 0.05). CSF protein levels were consistent across all groups (p = 0.1 and p = 0.7). Oligoclonal bands were detected in all patients with BD+MS, in the majority of MS patients (83.6 %), and a small percentage of pNBD patients (19.7 %), showing a notable distinction between the BD+MS and pNBD groups (p < 0.001).
CONCLUSIONS: Our study underscores the need for a skeptical approach in diagnosing and treating patients with BD who exhibit symptomatic MS-like MRI lesions. Our findings suggest that BD+MS is a distinct clinical entity, warranting specific diagnostic and treatment approaches. Our findings highlight that BD patients with MS-like lesions meeting MS diagnostic criteria should be managed as patients with comorbid MS and BD rather than pNBD.