Abstract：
OBJECTIVE: 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.
METHODS: 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.
RESULTS: 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 (χ2 = 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.
CONCLUSIONS: 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.
METHODS: 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.
RESULTS: 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 (χ2 = 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.
CONCLUSIONS: 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.
摘要：
目的：Wernicke脑病（WE）的临床诊断可能由于经典三联征的不完整表现而具有挑战性。目的是提供有关标准MRI相关性的最新信息，并将典型和非典型影像学发现与临床特征结合起来。
方法：在这项双中心回顾性观察研究中，我们在当地放射信息系统中搜索了临床或影像学怀疑为WE的连续患者.两名独立评估者评估了T2加权成像(WI)，流体衰减反演恢复(FLAIR),扩散WI(DWI)，T2*WI和/或磁化率WI(SWI)，和对比度增强（CE）-T1WI，并注意到典型的参与(即，乳头体（MB），导水管周围灰色（PAG），丘脑,下丘脑,盖板）和非典型（所有其他）病变部位。还记录了异常的信号模式，例如出血。报告的临床特征以及欧洲神经学会联合会（EFNS）最新指南的诊断标准用于测试与MRI生物标志物的关系。
结果：纳入了47例临床确诊的WE患者（Jan\'99-Apr\'23；平均年龄，53岁；70%男性）。影像学检查结果的中间可靠性很高（κ=0.71），与所有其他序列相比，T2WI（κ=0.85）的一致性最低，与所有其他典型区域相比，PAG（κ=0.65）的一致性最低。在共识中,77%(n=36/47)的WE病例被评为MRI阳性,与所有其他序列相比，FLAIR（n=36/47，77％）显示出最强的相关性（χ2=47.0；P<0.001）。在接受SWI的十分之四的患者中检测到MB中的微出血，在相应的T2*WI上不可见。在23％（n=11/47）的病例中观察到非典型发现，总是伴随着典型的发现，酗酒者(n=9/44，21%)和非酗酒者(n=2/3，67%)。孤立的结构参与，明确PAG(n=4/36;11%)或MB(n=1/36;3%)，存在，但观察到的频率低于合并病变（n=31/36；86％）。在病例与年龄和性别匹配的对照之间，在2D轴向FLAIR上PAG的截止宽度为2.5mm。仅在短期记忆丧失和MB变化之间证明了独立关联（OR=2.2[95％CI：1.1-4.5]；P=0.024）。回想起来，在每种情况下，EFNS标准均为阳性（4个中≥2个），但它的计数（范围，2-4）与标准MRI上的信号变化没有显着关系（P=0.427）。
结论：提出的序列协议(FLAIR，DWI,SWI和T1WICE）对WE的神经放射学检查结果具有良好的检出率，SWI显示MB中的微出血具有优异的可检测性。然而,大约四分之一病例的假阴性结果强调了神经系统警觉性对诊断的重要性。应提高对非典型MRI表现的认识，不仅在非酗酒者。临床体征和标准MRI生物标志物之间存在有限的相关性。
方法：在这项双中心回顾性观察研究中，我们在当地放射信息系统中搜索了临床或影像学怀疑为WE的连续患者.两名独立评估者评估了T2加权成像(WI)，流体衰减反演恢复(FLAIR),扩散WI(DWI)，T2*WI和/或磁化率WI(SWI)，和对比度增强（CE）-T1WI，并注意到典型的参与(即，乳头体（MB），导水管周围灰色（PAG），丘脑,下丘脑,盖板）和非典型（所有其他）病变部位。还记录了异常的信号模式，例如出血。报告的临床特征以及欧洲神经学会联合会（EFNS）最新指南的诊断标准用于测试与MRI生物标志物的关系。
结果：纳入了47例临床确诊的WE患者（Jan\'99-Apr\'23；平均年龄，53岁；70%男性）。影像学检查结果的中间可靠性很高（κ=0.71），与所有其他序列相比，T2WI（κ=0.85）的一致性最低，与所有其他典型区域相比，PAG（κ=0.65）的一致性最低。在共识中,77%(n=36/47)的WE病例被评为MRI阳性,与所有其他序列相比，FLAIR（n=36/47，77％）显示出最强的相关性（χ2=47.0；P<0.001）。在接受SWI的十分之四的患者中检测到MB中的微出血，在相应的T2*WI上不可见。在23％（n=11/47）的病例中观察到非典型发现，总是伴随着典型的发现，酗酒者(n=9/44，21%)和非酗酒者(n=2/3，67%)。孤立的结构参与，明确PAG(n=4/36;11%)或MB(n=1/36;3%)，存在，但观察到的频率低于合并病变（n=31/36；86％）。在病例与年龄和性别匹配的对照之间，在2D轴向FLAIR上PAG的截止宽度为2.5mm。仅在短期记忆丧失和MB变化之间证明了独立关联（OR=2.2[95％CI：1.1-4.5]；P=0.024）。回想起来，在每种情况下，EFNS标准均为阳性（4个中≥2个），但它的计数（范围，2-4）与标准MRI上的信号变化没有显着关系（P=0.427）。
结论：提出的序列协议(FLAIR，DWI,SWI和T1WICE）对WE的神经放射学检查结果具有良好的检出率，SWI显示MB中的微出血具有优异的可检测性。然而,大约四分之一病例的假阴性结果强调了神经系统警觉性对诊断的重要性。应提高对非典型MRI表现的认识，不仅在非酗酒者。临床体征和标准MRI生物标志物之间存在有限的相关性。
