Takahashi Sunao, Sanjo Nobuo, Miyamoto Shohei, Hattori Takaaki, Oyama Jun, Tateishi Ukihide, Yokota Takanori
Department of Neurology and Neurological Science, Tokyo Medical and Dental University, 1-5-45 Yushima Bunkyo-ku, Tokyo 113-8510, Japan.
Department of Neurology and Neurological Science, Tokyo Medical and Dental University, 1-5-45 Yushima Bunkyo-ku, Tokyo 113-8510, Japan; Department of General Internal Medicine, Asahi General Hospital, 1326, I, Asahi-shi, Chiba 289-2500, Japan.
J Neurol Sci. 2021 Feb 15;421:117284. doi: 10.1016/j.jns.2020.117284. Epub 2020 Dec 20.
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.
慢性进行性神经白塞病（CPNBD）的特征是认知功能缓慢进行性下降、小脑共济失调和脑干萎缩，无急性脑膜脑炎。为评估CPNBD在治疗期间的进展，我们对CPNBD患者的临床特征和脑磁共振成像（MRI）进行了一项回顾性纵向对比分析。我们将参与者分为三组：仅患有急性脑膜脑炎的白塞病（A组，8例急性神经白塞病[ANBD]患者）、原发性进行性CPNBD（B组，3例患者）以及急性脑膜脑炎与慢性进展并存（C组，2例患者）。对常规实验室检查、第三脑室宽度的每月扩大率（ΔWTVm）以及ΔWTVm与脑横径的相对值（ΔWTVIm）进行了统计学评估。虽然在ANBD中观察到脑脊液中的细胞计数较高以及白细胞介素-6浓度较高，但CPNBD期的ΔWTVm（p = 0.008）和ΔWTVIm（p = 0.008）均显著大于ANBD期。对CPNBD的有效治疗似乎可使部分患者的ΔWTVm和ΔWTVIm降低。对CPNBD患者的第三脑室宽度进行连续评估是早期诊断和治疗疗效的一种高敏感性候选生物标志物。
