ACCURACY OF CLINICAL-CRITERIA FOR THE DIAGNOSIS OF PROGRESSIVE SUPRANUCLEAR PALSY (STEELE-RICHARDSON-OLSZEWSKI SYNDROME)

We assessed the validity and interrater reliability of neurologists wh o, using four different sets of previously published criteria for the clinical diagnosis of progressive supranuclear palsy (PSP), also calle d Steele-Richardson-Olszewski syndrome, rated 105 autopsy-proven cases of PSP (n = 24), Lewy body disease (n = 29), corticobasal ganglionic degeneration (n = 10), postencephalitic parkinsonism (n = 7), multiple system atrophy (n = 16), Pick's disease (n = 7), and other parkinsoni an or dementia disorders (n = 12). Cases were presented in random orde r to six neurologists. Information from each patient's first and last visits to the medical center supplying the case was presented sequenti ally to the rater, and the rater's diagnosis was compared with the neu ropathologic diagnosis of each case. Interrater agreement for the diag nosis 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 identi fy the variables from the data set that would best predict the diagnos is. 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 t he regression analysis and the addition of exclusionary features, we p ropose optimal criteria for the clinical diagnosis of PSP.