Chronic progressive neuro-Behçet's disease (CPNBD) is characterized by slowly progressive cognitive decline, cerebellar ataxia, and brainstem atrophy without acute encephalomeningitis. To evaluate the progression of CPNBD during treatment, we conducted a retrospective, longitudinal comparative analysis of the clinical features and brain magnetic resonance imaging (MRI) in patients with CPNBD. We classified participants into three groups: NBD with acute encephalomeningitis alone (Group A, 8 patients with acute neuro-Behçet's disease [ANBD]), primary progressive CPNBD (Group B, 3 patients), and a combination of acute encephalomeningitis, and chronic progression (Group C, 2 patients). Routine laboratory tests and monthly rate of enlargement of the width of the third ventricle (ΔWTVm) and relative value of ΔWTVm to the transverse cerebral diameter (ΔWTVIm) were statistically evaluated. Although higher cell count values and interleukin-6 concentration in the cerebrospinal fluid were observed in ANBD, both ΔWTVm (p = 0.008) and ΔWTVIm (p = 0.008) were significantly larger in CPNBD phase than in the ANBD phase. Effective treatment for CPNBD seemed to reduce ΔWTVm and ΔWTVIm in some patients. Sequential evaluation of WTV in patients with CPNBD is a highly sensitive candidate biomarker of early diagnosis and treatment efficacy.

中文翻译：

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第三脑室宽度作为慢性进行性神经白塞病的高度敏感生物标志物

慢性进行性神经白塞病 (CPNBD) 的特征是缓慢进行性认知能力下降、小脑共济失调和脑干萎缩，但没有急性脑膜炎。为了评估治疗期间 CPNBD 的进展，我们对 CPNBD 患者的临床特征和脑磁共振成像 (MRI) 进行了回顾性纵向比较分析。我们将参与者分为三组：仅伴有急性脑膜炎的 NBD（A 组，8 名急性神经白塞病 [ANBD] 患者）、原发性进展性 CPNBD（B 组，3 名患者）以及急性脑膜炎和慢性进展的组合（C组，2名患者）。对常规实验室检查和每月第三脑室宽度扩大率 (ΔWTVm) 和 ΔWTVm 与大脑横径 (ΔWTVIm) 的相对值进行统计评估。尽管在 ANBD 中观察到脑脊液中更高的细胞计数值和白细胞介素 6 浓度，但 CPNBD 期的 ΔWTVm（p = 0.008）和 ΔWTVIm（p = 0.008）均显着大于 ANBD 期。CPNBD 的有效治疗似乎可以降低某些患者的 ΔWTVm 和 ΔWTVIm。CPNBD 患者 WTV 的顺序评估是早期诊断和治疗效果的高度敏感的候选生物标志物。008) 在 CPNBD 阶段显着大于在 ANBD 阶段。CPNBD 的有效治疗似乎可以降低某些患者的 ΔWTVm 和 ΔWTVIm。CPNBD 患者 WTV 的顺序评估是早期诊断和治疗效果的高度敏感的候选生物标志物。008) 在 CPNBD 阶段显着大于在 ANBD 阶段。CPNBD 的有效治疗似乎可以降低某些患者的 ΔWTVm 和 ΔWTVIm。CPNBD 患者 WTV 的顺序评估是早期诊断和治疗效果的高度敏感的候选生物标志物。