Institute of Neuroradiology, University Hospital Frankfurt, Goethe University, Frankfurt/Main, Germany.
Department of Neurology, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany.
Clin Neuroradiol. 2024 Dec;34(4):881-897. doi: 10.1007/s00062-024-01434-y. Epub 2024 Jul 16.
Clinical diagnosis of Wernicke encephalopathy (WE) can be challenging due to incomplete presentation of the classical triad. The aim was to provide an update on the relevance of standard MRI and to put typical and atypical imaging findings into context with clinical features.
In this two-center retrospective observational study, the local radiology information system was searched for consecutive patients with clinical or imaging suspicion of WE. Two independent raters evaluated T2-weighted imaging (WI), fluid-attenuation inversion recovery (FLAIR), diffusion WI (DWI), T2*WI and/or susceptibility WI (SWI), and contrast-enhanced (CE)-T1WI, and noted the involvement of typical (i.e., mammillary bodies (MB), periaqueductal grey (PAG), thalamus, hypothalamus, tectal plate) and atypical (all others) lesion sites. Unusual signal patterns like hemorrhages were also documented. Reported clinical features together with the diagnostic criteria of the latest guidelines of the European Federation of Neurological Societies (EFNS) were used to test for relationships with MRI biomarkers.
47 patients with clinically confirmed WE were included (Jan '99-Apr '23; mean age, 53 yrs; 70% males). Interrater reliability for imaging findings was substantial (κ = 0.71), with lowest agreements for T2WI (κ = 0.85) compared to all other sequences and for PAG (κ = 0.65) compared to all other typical regions. In consensus, 77% (n = 36/47) of WE cases were rated MRI positive, with FLAIR (n = 36/47, 77%) showing the strongest relation (χ = 47.0; P < 0.001) compared to all other sequences. Microbleeds in the MB were detected in four out of ten patients who received SWI, not visible on corresponding T2*WI. Atypical findings were observed in 23% (n = 11/47) of cases, always alongside typical findings, in both alcoholics (n = 9/44, 21%) and non-alcoholics (n = 2/3, 67%). Isolated involvement of structures, explicitly PAG (n = 4/36; 11%) or MB (n = 1/36; 3%), was present but observed less frequently than combined lesions (n = 31/36; 86%). A cut-off width of 2.5 mm for the PAG on 2D axial FLAIR was established between cases and age- and sex-matched controls. An independent association was demonstrated only between short-term memory loss and changes in the MB (OR = 2.2 [95% CI: 1.1-4.5]; P = 0.024). In retrospect, EFNS criteria were positive (≥ 2 out of 4) in every case, but its count (range, 2-4) showed no significant (P = 0.427) relationship with signal changes on standard MRI.
The proposed sequence protocol (FLAIR, DWI, SWI and T1WI + CE) yielded good detection rates for neuroradiological findings in WE, with SWI showing microbleeds in the MB with superior detectability. However, false negative results in about a quarter of cases underline the importance of neurological alertness for the diagnosis. Awareness of atypical MRI findings should be raised, not only in non-alcoholics. There is limited correlation between clinical signs and standard MRI biomarkers.
由于不完全呈现经典三联征,威尼克脑病(WE)的临床诊断具有挑战性。本研究旨在提供标准 MRI 的相关性更新,并将典型和非典型影像学表现与临床特征联系起来。
在这项两中心回顾性观察性研究中,对当地放射学信息系统进行了搜索,以查找具有 WE 临床或影像学怀疑的连续患者。两名独立的评估者评估了 T2 加权成像(WI)、液体衰减反转恢复(FLAIR)、弥散 WI(DWI)、T2*WI 和/或磁化率 WI(SWI)以及对比增强(CE)-T1WI,并记录了典型(即乳头部(MB)、导水管周围灰质(PAG)、丘脑、下丘脑、脑桥盖)和非典型(所有其他)病变部位的受累情况。还记录了异常信号模式,如出血。报告的临床特征以及欧洲神经病学学会联合会(EFNS)最新指南的诊断标准用于测试与 MRI 生物标志物的关系。
包括 47 例经临床证实的 WE 患者(1999 年 1 月至 2023 年 4 月;平均年龄 53 岁;70%为男性)。影像学发现的组内一致性很高(κ=0.71),与所有其他序列相比,T2WI 的一致性最低(κ=0.85),与所有其他典型区域相比,PAG 的一致性最低(κ=0.65)。在共识中,77%(n=36/47)的 WE 病例被评为 MRI 阳性,FLAIR(n=36/47,77%)与所有其他序列相比具有最强的相关性(χ²=47.0;P<0.001)。在接受 SWI 的十名患者中,有四名患者检测到 MB 中的微出血,但在相应的 T2*WI 上不可见。23%(n=11/47)的病例观察到非典型表现,总是与典型表现一起出现,在酒精中毒患者(n=9/44,21%)和非酒精中毒患者(n=2/3,67%)中均观察到。单独累及结构,明确为 PAG(n=4/36;11%)或 MB(n=1/36;3%)的情况也存在,但比合并病变(n=31/36;86%)少见。在 2D 轴位 FLAIR 上,PAG 的宽度为 2.5mm 被确立为病例与年龄和性别匹配的对照组之间的界限。仅在短期记忆丧失和 MB 变化之间显示出独立的关联(OR=2.2 [95%CI:1.1-4.5];P=0.024)。回顾性分析表明,EFNS 标准在每种情况下均为阳性(≥2/4),但其计数(范围,2-4)与标准 MRI 上的信号变化无显著相关性(P=0.427)。
提出的序列方案(FLAIR、DWI、SWI 和 T1WI+CE)对 WE 的神经影像学发现具有良好的检出率,SWI 显示 MB 中的微出血具有更高的可检测性。然而,大约四分之一的病例出现假阴性结果,这突显了对诊断保持神经警觉的重要性。应提高对非酒精中毒患者非典型 MRI 表现的认识。临床体征与标准 MRI 生物标志物之间存在有限的相关性。