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 at the University of California, San Francisco, Department of Neurology, University of California, San Francisco.
JAMA Netw Open. 2022 Apr 1;5(4):e229588. doi: 10.1001/jamanetworkopen.2022.9588.
The accurate diagnosis of progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) is hampered by imperfect clinical-pathological correlations.
To assess and compare the diagnostic value of the magnetic resonance parkinsonism index (MRPI) and other magnetic resonance imaging-based measures of cerebral atrophy to differentiate between PSP, CBD, and other neurodegenerative diseases.
DESIGN, SETTING, AND PARTICIPANTS: This prospective diagnostic study included participants with 4-repeat tauopathies (4RT), PSP, CBD, other neurodegenerative diseases and available MRI who appeared in the University of California, San Francisco, Memory and Aging Center database. Data were collected from October 27, 1994, to September 29, 2019. Data were analyzed from March 1 to September 14, 2021.
The main outcome of this study was the neuropathological diagnosis of PSP or CBD. The clinical diagnosis at the time of the MRI acquisition was noted. The imaging measures included the MRPI, cortical thickness, subcortical volumes, including the midbrain, pons, and superior cerebellar peduncle volumes. Multinomial logistic regression models (MLRM) combining different cortical and subcortical regions were defined to discriminate between PSP, CBD, and other pathologies. The areas under the receiver operating characteristic curves (AUROC) and cutoffs were calculated to differentiate between PSP, CBD, and other diseases.
Of the 326 included participants, 176 (54%) were male, and the mean (SD) age at MRI was 64.1 (8.0) years. The MRPI showed good diagnostic accuracy for the differentiation between PSP and all other pathologies (accuracy, 87%; AUROC, 0.90; 95% CI, 0.86-0.95) and between 4RT and other pathologies (accuracy, 80%; AUROC, 0.82; 95% CI, 0.76-0.87), but did not allow the discrimination of participants with CBD. Its diagnostic accuracy was lower in the subgroup of patients without the canonical PSP-Richardson syndrome (PSP-RS) or probable corticobasal syndrome (CBS) at MRI. MLRM combining cortical and subcortical measurements showed the highest accuracy for the differentiation between PSP and other pathologies (accuracy, 95%; AUROC, 0.98; 95% CI, 0.97-0.99), CBD and other pathologies (accuracy, 83%; AUROC, 0.86; 95% CI, 0.81-0.91), 4RT and other pathologies (accuracy, 89%; AUROC, 0.94; 95% CI, 0.92-0.97), and PSP and CBD (accuracy, 91%; AUROC, 0.95; 95% CI, 0.91-0.99), even in participants without PSP-RS or CBS at MRI.
In this study, the combination of widely available cortical and subcortical measures of atrophy on MRI discriminated between PSP, CBD, and other pathologies and could be used to support the diagnosis of 4RT in clinical practice.
进行性核上性麻痹（PSP）和皮质基底节变性（CBD）的准确诊断受到临床-病理相关性不完善的阻碍。
评估和比较磁共振帕金森病指数（MRPI）和其他基于磁共振成像的脑萎缩测量值在区分 PSP、CBD 和其他神经退行性疾病方面的诊断价值。
设计、地点和参与者：本前瞻性诊断研究纳入了具有 4 重复 tau 病变（4RT）、PSP、CBD、其他神经退行性疾病和可用 MRI 的参与者，这些参与者出现在加利福尼亚大学旧金山分校记忆与衰老中心数据库中。数据收集于 1994 年 10 月 27 日至 2019 年 9 月 29 日。数据分析于 2021 年 3 月 1 日至 9 月 14 日进行。
本研究的主要结果是 PSP 或 CBD 的神经病理学诊断。在 MRI 采集时记录了临床诊断。影像学测量包括 MRPI、皮质厚度、包括中脑、脑桥和上小脑脚体积在内的皮质下体积。定义了结合不同皮质和皮质下区域的多项逻辑回归模型（MLRM），以区分 PSP、CBD 和其他病变。计算了受试者工作特征曲线下面积（AUROC）和截断值，以区分 PSP、CBD 和其他疾病。
在纳入的 326 名参与者中，176 名（54%）为男性，MRI 时的平均（SD）年龄为 64.1（8.0）岁。MRPI 对区分 PSP 和所有其他病变（准确性为 87%；AUROC，0.90；95%CI，0.86-0.95）和 4RT 与其他病变（准确性为 80%；AUROC，0.82；95%CI，0.76-0.87）具有良好的诊断准确性，但不能区分 CBD 患者。在没有 MRI 上典型 PSP-Richardson 综合征（PSP-RS）或可能的皮质基底节综合征（CBS）的患者亚组中，其诊断准确性较低。结合皮质和皮质下测量的 MLRM 对区分 PSP 和其他病变（准确性，95%；AUROC，0.98；95%CI，0.97-0.99）、CBD 和其他病变（准确性，83%；AUROC，0.86；95%CI，0.81-0.91）、4RT 和其他病变（准确性，89%；AUROC，0.94；95%CI，0.92-0.97）、PSP 和 CBD（准确性，91%；AUROC，0.95；95%CI，0.91-0.99）的准确性最高，甚至在 MRI 上没有 PSP-RS 或 CBS 的患者中也是如此。
在这项研究中，磁共振成像上广泛可用的皮质和皮质下萎缩测量值的组合可区分 PSP、CBD 和其他病变，并可用于支持临床实践中 4RT 的诊断。
