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

Abstract

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 = 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 parkinsonian or dementia disorders (n = 12). Cases were presented in random order to six neurologists. Information from each patient's first and last visits to the medical center supplying the case was presented sequentially to the rater, and the rater's 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.

Differential Diagnosis of Parkinsonism Using Dual-Phase F-18 FP-CIT PET Imaging

“…The diagnosis of IPD was based on the UK Parkinson's Disease Society Brain Bank Clinical Diagnostic Criteria [22]. Patients with clinically probable MSA-P, MSA-C, PSP, and DLB were enrolled based on current diagnostic criteria [4,23,24]. All patients were assessed after at least a 1-year clinical follow-up by a neurologist specializing in movement disorders.…”

“…Idiopathic Parkinson's disease (IPD) is the most prevalent cause [2]; however, approximately one-third of patients with parkinsonian symptoms have another disease, particularly atypical parkinsonism (APD), such as multiple-system atrophy (MSA), progressive supranuclear palsy (PSP), and dementia with Lewy bodies (DLB) [3]. There are several clinical clues that suggest APD [4,5]. However, the differential diagnosis of parkinsonism continues to be challenging with a high misdiagnosis rate, particularly in the early stage [6], because parkinsonian patients show similar symptoms and specific symptoms do not appear in early stage [7].…”

Frontotemporal Dementia: Implications for Understanding Alzheimer Disease

“…The clinical presentation of PSP includes vertical supranuclear ophtalmoplegia with difficulty looking up, bradykinesia, axial dystonia and rigidity, pseudobulbar palsy and postural instability with backward falls (Steele et al 1964;Litvan et al 1996). More than half of the patients develop cognitive impairment.…”

An uncommon presentation of Progressive Supranuclear Palsy diagnosed on DaT scan

“…Apathy, disinhibition, dysphoria, and anxiety are common features of PSP. Characteristic clinical signs include a vertical supranuclear palsy with downward gaze abnormalities and postural instability with unexplained falls [1][2]. Bradykinesia and rigidity are typically symmetrical in onset.…”