Accuracy of the clinical diagnosis of corticobasal degeneration: A clinicopathologic study
Accuracy of the clinical diagnosis of corticobasal degeneration: A clinicopathologic study
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DOI：
10.1212/wnl.48.1.119
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发表时间：
1997-01-01
期刊：
影响因子：
9.9
通讯作者：
Bartko, JJ
中科院分区：
文献类型：
--
作者：
Litvan, I;Agid, Y;Bartko, JJ
皮质基底节变性（CBD）的临床诊断的准确性是未知的。为了确定其诊断的准确性，我们提出了105例已知的神经病理诊断，包括CBD（n = 10），进行性核上性麻痹，（PSP，n = 24），帕金森病（n = 15），弥漫性路易体病（n = 14），多系统萎缩（n = 16），脑炎后帕金森综合征（n = 7），皮克病（n = 7），Creutzfeldt-Jacob病（n = 4）、阿尔茨海默病（n = 4）、血管性帕金森综合征（n = 3）和维普莱病（n = 1），作为6名不知道尸检结果的神经科医生的临床小插曲。可靠性用Kappa统计量进行测量。在首次和末次临床访视时，将神经科医生的临床诊断与临床病理诊断的敏感性、特异性和阳性预测值进行比较。诊断CBD的组可靠性显著提高，从第一次就诊的中度（平均=发病后34个月）到最后一次就诊的显著（发病后68个月）。对于第一次访问，CBD的平均灵敏度较低（35%），但特异性接近完美（99.6%）。对于最后一次访视，平均灵敏度略有增加（48.3%），特异性保持稳定。假阴性误诊主要发生在PSP。假阳性诊断是罕见的。CBD临床诊断的敏感性极低，表明这种疾病明显诊断不足。虽然如果神经科医生能够检查这些患者，临床诊断的有效性可能会得到提高，但更重要的是，这种疾病被初级神经科医生误诊。在我们的数据集中，诊断CBD的最佳预测因素包括肢体肌张力障碍、识别性失用症、肌阵挛和迟发性步态或平衡障碍的不对称运动僵硬综合征。
The accuracy of the clinical diagnosis of corticobasal degeneration (CBD) is unknown. To determine its diagnostic accuracy, we presented 105 cases with known neuropathologic diagnoses, including CBD (n = 10), progressive supranuclear palsy (PSP, n = 24), Parkinson's disease (n = 15), diffuse Lewy body disease (n = 14), multiple system atrophy (n = 16), postencephalitic parkinsonism (n = 7), Pick's disease (n = 7), Creutzfeldt-Jacob disease (n = 4), Alzheimer's disease (n = 4), vascular parkinsonism (n = 3), and Whipple's disease (n = 1), as clinical vignettes to six neurologists unaware of the autopsy findings. Reliability was measured with the kappa statistics. The neurologists' clinical diagnoses were compared with clinicopathologic diagnoses for sensitivity, specificity, and positive predictive values at first and last clinic visits. The group reliability for the diagnosis of CBD significantly improved from moderate for the first visit (mean = 34 months after onset) to substantial for the last (68 months after onset). For the first visit, mean sensitivity for CBD was low (35%), but specificity was near-perfect (99.6%). For the last visit, mean sensitivity minimally increased (48.3%), and specificity remained stable. False-negative misdiagnoses mainly occurred with PSP. False-positive diagnoses were rare. The extremely low sensitivity of the clinical diagnosis of CBD suggests that this disorder is markedly underdiagnosed. Although the validity of the clinical diagnosis might have been improved if neurologists could have examined these patients, more important is that this disorder was misdiagnosed by the primary neurologists. In our data set, the best predictors for the diagnosis of CBD included limb dystonia, ideomotor apraxia, myoclonus, and asymmetric akinetic-rigid syndrome with late onset of gait or balance disturbances.