Sant Pau Memory Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain.
Atlantic Fellow for Equity in Brain Health, Department of Neurology, University of California, San Francisco.
JAMA Netw Open. 2021 Mar 1;4(3):e211290. doi: 10.1001/jamanetworkopen.2021.1290.
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).
DESIGN, SETTING, AND PARTICIPANTS: 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.
磁共振成像上的萎缩存在可以支持行为变异额颞叶痴呆（bvFTD）的诊断，但缺乏可重复的测量。
评估 6 种视觉萎缩量表（VAS）和磁共振帕金森指数（MRPI）的诊断和预后效用。
设计、设置和参与者：在这项诊断/预后研究中，来自 3 个中心的 235 名 bvFTD 患者和 225 名年龄和磁共振成像匹配的对照个体的数据于 1998 年 12 月 1 日至 2019 年 9 月 30 日收集。121 名 bvFTD 患者具有较高的额颞叶变性（FTLD）置信度（bvFTD-HC），19 名具有较低的 FTLD 置信度（bvFTD-LC）。盲法临床医生应用了 6 种先前验证过的 VAS，并用全自动方法计算了 MRPI。还比较了皮质厚度和皮质下体积的测量值。数据分析于 2020 年 2 月 1 日至 6 月 30 日进行。
本研究的主要结果是 bvFTD-HC 或 4 重复（4R）tau 病变的神经病理学诊断和临床恶化率（通过临床痴呆评定量表总和框的纵向测量评估）。脑萎缩的测量指标包括 VAS 评分、bvFTD 萎缩评分（眶额、前扣带回、前颞叶、内侧颞叶和额极叶区域的 VAS 评分总和）、MRPI 以及皮质和皮质下体积的其他计算机量化。计算了受试者工作特征曲线（ROC）的曲线下面积（AUROC），以区分 bvFTD-HC 和 bvFTD-LC 以及对照组的患者。线性混合模型用于评估萎缩测量值估计纵向临床恶化的能力。
在 460 名纳入的参与者中，296 名（64.3%）为男性，平均（SD）年龄为 62.6（11.4）岁。bvFTD 萎缩评分对区分 bvFTD-HC 与对照组（AUROC，0.930；95%CI，0.903-0.957）和 bvFTD-HC 与 bvFTD-LC（AUROC，0.880；95%CI，0.787-0.972）的准确性与计算机测量值相当（AUROC，0.973 [95%CI，0.954-0.993]和 0.898 [95%CI，0.834-0.962]）。与其他 FTLD 亚型相比，bvFTD 患者和潜在的 4R tau 病变患者的 MRPI 增加（14.1 [2.0] 与 11.2 [2.6] 点；P < .001）。bvFTD 萎缩评分较高与 bvFTD 临床恶化较快相关（bvFTD 萎缩评分每年增加 1.86 分，临床痴呆评定量表总和框评分增加 1 分；95%CI，0.99-2.73；P < .001）。
基于这些研究结果，在 bvFTD 中，VAS 增加了潜在 FTLD 的诊断确定性，MRPI 显示出检测潜在 4R tau 病变患者的潜力。这些广泛可用的萎缩测量方法也可用于估计纵向临床恶化。
