Accuracy of clinical criteria for the diagnosis of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome)

作者：Irene Litvan, Yves Agid, Joseph J. Jankovic, Christopher G. Goetz, J.-P. Brandel, Eugene C. Lai, Gregor Karl Wenning, Luis D'Olhaberriague, Marc Verny, К. Ray Chaudhuri, Ann Carolyn McKee, K. A. Jellinger, John J. Bartko, Carlos Alberto Mangone, R. K. B. Pearce · 发表于：Neurology · 年份：1996 · DOI：10.1212/wnl.46.4.922 · 被引用次数：349 · 研究领域：Parkinson's Disease Mechanisms and Treatments、Neurological disorders and treatments、Autism Spectrum Disorder Research

We assessed the validity and interrater reliability of neurologists who, using four different sets of previously published criteria for the clinical diagnosis of progressive supranuclear palsy (PSP), also called Steele-Richardson-Olszewski syndrome, rated 105 autopsy-proven cases of PSP (n equals 24), Lewy body disease (n equals 29), corticobasal ganglionic degeneration (n equals 10), postencephalitic parkinsonism (n equals 7), multiple system atrophy (n equals 16), Pick9s disease (n equals 7), and other parkinsonian or dementia disorders (n equals 12). Cases were presented in random order to six neurologists. Information from each patient9s first and last visits to the medical center supplying the case was presented sequentially to the rater, and the rater9s diagnosis was compared with the neuropathologic diagnosis of each case. Interrater agreement for the diagnosis of PSP varied from substantial to near perfect, but none of the criteria had both high sensitivity and high predictive value. Because of these limitations, we used a logistic regression analysis to identify the variables from the data set that would best predict the diagnosis. This analysis identified vertical supranuclear palsy with downward gaze abnormalities and postural instability with unexplained falls as the best features for predicting the diagnosis. From the results of the regression analysis and the addition of exclusionary features, we propose optimal criteria for the clinical diagnosis of PSP. NEUROL...