Diagnostic Utility of Measuring Cerebral Atrophy in the Behavioral Variant of Frontotemporal Dementia and Association With Clinical Deterioration.

Diagnostic Utility of Measuring Cerebral Atrophy in the Behavioral Variant of Frontotemporal Dementia and Association With Clinical Deterioration.
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DOI:
10.1001/jamanetworkopen.2021.1290
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发表时间:
2021-03-01
期刊:
影响因子:
13.8
通讯作者:
Perry DC
Perry DC
中科院分区:
医学1区
文献类型:
--
作者:
Illán-Gala I;Falgàs N;Friedberg A;Castro-Suárez S;Keret O;Rogers N;Oz D;Nigro S;Quattrone A;Quattrone A;Wolf A;Younes K;Santos-Santos M;Borrego-Écija S;Cobigo Y;Dols-Icardo O;Lladó A;Sánchez-Valle R;Clarimon J;Blesa R;Alcolea D;Fortea J;Lleó A;Grinberg LT;Spina S;Kramer JH;Rabinovici GD;Boxer A;Gorno Tempini ML;Miller BL;Seeley WW;Rosen HJ;Perry DC

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磁共振成像上广泛可用的萎缩测量是否可以增加潜在额颞叶变性(FTLD)的诊断确定性并估计额颞叶痴呆行为变体(bvFTD)的临床恶化?本诊断/预后研究调查了5种经验证的视觉萎缩量表(VAS)和磁共振帕金森综合征指数的临床效用。当合并时,VAS显示出区分FTLD高置信度和低置信度bvFTD以及估计纵向临床恶化的极好诊断性能,而在基础4-重复tau蛋白病的bvFTD中,磁共振帕金森病指数升高。这些结果表明,在bvFTD中,VAS可用于增加基础FTLD的诊断确定性并估计纵向临床恶化。本诊断/预后研究评估了6种视觉萎缩量表和磁共振帕金森综合征指数在行为变异型额颞叶痴呆患者中的实用性,以区分额颞叶变性的高置信度与低置信度。磁共振成像上萎缩的存在可以支持额颞叶痴呆(bvFTD)的行为变异的诊断,但缺乏可重复的测量。评估6种视觉萎缩量表(VAS)和磁共振帕金森综合征指数(MRPI)的诊断和预后效用。在这项诊断/预后研究中,从1998年12月1日至2019年9月30日收集了来自3个中心的235例bvFTD患者和225例年龄和磁共振成像匹配的对照个体的数据。121例bvFTD受试者的额颞叶变性(FTLD)置信度较高(bvFTD-HC),19例FTLD置信度较低(bvFTD-LC)。设盲的临床医生应用6个先前验证的VAS,并使用全自动方法计算MRPI。皮质厚度和皮质下体积也进行了测量进行比较。数据分析时间为2020年2月1日至6月30日。本研究的主要结局是bvFTD-HC或4-重复(4 R)tau蛋白病的神经病理学诊断和临床恶化率(通过临床痴呆评定总和的纵向测量进行评估)。脑萎缩指标包括VAS评分、bvFTD萎缩评分(眶额、前扣带回、前颞叶、内侧颞叶和额叶皮质区的VAS评分总和)、MRPI和皮质和皮质下体积的其他计算机定量。计算受试者工作特征曲线下面积(AUROC),以区分bvFTD-HC和bvFTD-LC受试者与对照组。线性混合模型用于评估萎缩措施估计纵向临床恶化的能力。在纳入的460名参与者中,296名(64.3%)为男性,平均(SD)年龄为62.6(11.4)岁。bvFTD萎缩评分区分bvFTD-HC与对照的准确性(AUROC,0.930; 95% CI,0.903-0.957)和bvFTD-LC中的bvFTD-HC(AUROC,0.880; 95% CI,0.787-0.972)与计算机测量值(AUROC,分别为0.973 [95% CI,0.954-0.993]和0.898 [95% CI,0.834-0.962])相当。与其他FTLD亚型相比,bvFTD和基础4 R tau蛋白病患者的MRPI升高(14.1 [2.0] vs 11.2 [2.6]分; P <0.001)。bvFTD萎缩评分越高,bvFTD临床恶化越快(临床痴呆评定总分随bvFTD萎缩评分每年增加而变化1.86分; 95% CI,0.99-2.73; P <0.001)。基于这些研究结果,在bvFTD中,VAS增加了基础FTLD的诊断确定性,MRPI显示出检测基础4 R tau蛋白病受试者的潜力。这些广泛可用的萎缩指标也可用于估计纵向临床恶化。
Can widely available measures of atrophy on magnetic resonance imaging increase diagnostic certainty of underlying frontotemporal lobar degeneration (FTLD) and estimate clinical deterioration in the behavioral variant of frontotemporal dementia (bvFTD)? This diagnostic/prognostic study investigated the clinical utility of 5 validated visual atrophy scales (VAS) and the Magnetic Resonance Parkinsonism Index. When combined, VAS showed excellent diagnostic performance for differentiating between bvFTD with high and low confidence of FTLD and for the estimation of longitudinal clinical deterioration, whereas the Magnetic Resonance Parkinsonism Index was increased in bvFTD with underlying 4-repeat tauopathies. These findings suggest that, in bvFTD, VAS can be used to increase diagnostic certainty of underlying FTLD and estimate longitudinal clinical deterioration. This diagnostic/prognostic study assesses the utility of 6 visual atrophy scales and the Magnetic Resonance Parkinsonism Index in patients with behavioral variant frontotemporal dementia to distinguish those with high vs low confidence of frontotemporal lobar degeneration. The presence of atrophy on magnetic resonance imaging can support the diagnosis of the behavioral variant of frontotemporal dementia (bvFTD), but reproducible measurements are lacking. To assess the diagnostic and prognostic utility of 6 visual atrophy scales (VAS) and the Magnetic Resonance Parkinsonism Index (MRPI). In this diagnostic/prognostic study, data from 235 patients with bvFTD and 225 age- and magnetic resonance imaging–matched control individuals from 3 centers were collected from December 1, 1998, to September 30, 2019. One hundred twenty-one participants with bvFTD had high confidence of frontotemporal lobar degeneration (FTLD) (bvFTD-HC), and 19 had low confidence of FTLD (bvFTD-LC). Blinded clinicians applied 6 previously validated VAS, and the MRPI was calculated with a fully automated approach. Cortical thickness and subcortical volumes were also measured for comparison. Data were analyzed from February 1 to June 30, 2020. The main outcomes of this study were bvFTD-HC or a neuropathological diagnosis of 4-repeat (4R) tauopathy and the clinical deterioration rate (assessed by longitudinal measurements of Clinical Dementia Rating Sum of Boxes). Measures of cerebral atrophy included VAS scores, the bvFTD atrophy score (sum of VAS scores in orbitofrontal, anterior cingulate, anterior temporal, medial temporal lobe, and frontal insula regions), the MRPI, and other computerized quantifications of cortical and subcortical volumes. The areas under the receiver operating characteristic curve (AUROC) were calculated for the differentiation of participants with bvFTD-HC and bvFTD-LC and controls. Linear mixed models were used to evaluate the ability of atrophy measures to estimate longitudinal clinical deterioration. Of the 460 included participants, 296 (64.3%) were men, and the mean (SD) age was 62.6 (11.4) years. The accuracy of the bvFTD atrophy score for the differentiation of bvFTD-HC from controls (AUROC, 0.930; 95% CI, 0.903-0.957) and bvFTD-HC from bvFTD-LC (AUROC, 0.880; 95% CI, 0.787-0.972) was comparable to computerized measures (AUROC, 0.973 [95% CI, 0.954-0.993] and 0.898 [95% CI, 0.834-0.962], respectively). The MRPI was increased in patients with bvFTD and underlying 4R tauopathies compared with other FTLD subtypes (14.1 [2.0] vs 11.2 [2.6] points; P < .001). Higher bvFTD atrophy scores were associated with faster clinical deterioration in bvFTD (1.86-point change in Clinical Dementia Rating Sum of Boxes score per bvFTD atrophy score increase per year; 95% CI, 0.99-2.73; P < .001). Based on these study findings, in bvFTD, VAS increased the diagnostic certainty of underlying FTLD, and the MRPI showed potential for the detection of participants with underlying 4R tauopathies. These widely available measures of atrophy can also be useful to estimate longitudinal clinical deterioration.
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