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Prognostic predictors have not been defined for progressive supranuclear palsy (PSP) and multiple system atrophy (MSA). Subtypes of both disorders have been proposed on the basis of early clinical features. We performed a retrospective chart review to investigate the natural history of pathologically confirmed cases of PSP and MSA. Survival data and several clinically relevant milestones, namely: frequent falling, cognitive disability, unintelligible speech, severe dysphagia, dependence on wheelchair for mobility, the use of urinary catheters and placement in residential care were determined. On the basis of early symptoms, we subdivided cases with PSP into 'Richardson's syndrome' (RS) and 'PSP-parkinsonism' (PSP-P). Cases of MSA were subdivided according to the presence or absence of early autonomic failure. Sixty-nine (62.7%) of the 110 PSP cases were classified as RS and 29 (26.4%) as PSP-P. Of the 83 cases of MSA, 42 (53.2%) had autonomic failure within 2 years of disease onset. Patients with PSP had an older age of onset (P < 0.001), but similar disease duration to those with MSA. Patients with PSP reached their first clinical milestone earlier than patients with MSA (P < 0.001). Regular falls (P < 0.001), unintelligible speech (P = 0.04) and cognitive impairment (P = 0.03) also occurred earlier in PSP than in MSA. In PSP an RS phenotype, male gender, older age of onset and a short interval from disease onset to reaching the first clinical milestone were all independent predictors of shorter disease duration to death. Patients with RS also reached clinical milestones after a shorter interval from disease onset, compared to patients with PSP-P. In MSA early autonomic failure, female gender, older age of onset, a short interval from disease onset to reaching the first clinical milestone and not being admitted to residential care were independent factors predicting shorter disease duration until death. The time to the first clinical milestone is a useful prognostic predictor for survival. We confirm that RS had a less favourable course than PSP-P, and that early autonomic failure in MSA is associated with shorter survival. [ABSTRACT FROM AUTHOR]

Smell identification tests (SITs) have been suggested as useful in the differential diagnosis of Parkinson's disease (PD). We have applied the 40 item University of Pennsylvania SIT (UPSIT-40) and/or the 16 item SIT from Sniffin Sticks (SS-16) to 193 nondemented PD patients and 157 controls and used logistic regression analysis to associate the SIT result with the probability of an individual patient having PD ("PD probability"). Reliability measures (95% CI) using the clinical diagnosis as a gold standard and a dichotomized result of the smell test into high (50% or more) or low (<50%) "PD probability" were: sensitivity 85.0% (78.8-89.7%), specificity 84.6% (77.3-89.9%) for the UPSIT-40; sensitivity 90.4% (83.5-94.7%), specificity 85.5% (76.2-91.7%) for the SS-16. Based on these findings we have created color coded visual tools (PD probability rulers) and applied them to 39 clinically uncertain parkinsonian syndromes (CUPS) patients who had been investigated with dopamine transporter SPECT scanning using [123-I]-FP-CIT SPECT (DaTSCAN) for suspected Parkinson's disease. In 32 of 36 CUPS cases (88.9%, kappa = 0.72) the probability ruler predicted the result of the DaTSCAN. We suggest smell tests could be used routinely in challenging cases where there is diagnostic uncertainty and help inform decision making relating to the need for neuro imaging. [ABSTRACT FROM AUTHOR]

In five experiments, we investigated the speed of pitch resolution in a musical context. In experiments 1-3, listeners were presented an incomplete scale (doh, re, mi, fa, sol, la, ti) and then a probe tone. Listeners were instructed to make a rapid key-press response to probe tones that were relatively proximal in pitch to the last note of the scale (valid trials), and to ignore other probe tones (invalid trials). Reaction times were slower if the pitch of the probe tone was dissonant with the expected pitch (i.e., the completion of the scale, or doh) or if the probe tone was nondiatonic to the key implied by the scale. In experiments 4 and 5, listeners were presented a two-octave incomplete arpeggio, and then a probe tone. In this case, listeners were asked to make a rapid key-press response to probe tones that were relatively distant in pitch from the last note of the arpeggio. Under these conditions, registral direction and pitch proximity were the dominant influences on reaction time. Results are discussed in view of research on auditory attention and models of musical pitch. © 2001 Acoustical Society of America. [ABSTRACT FROM AUTHOR]