Clinical outcomes of progressive supranuclear palsy and multiple system atrophy
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Open Access
- 2 April 2008
- journal article
- research article
- Published by Oxford University Press (OUP) in Brain
- Vol. 131 (5), 1362-1372
- https://doi.org/10.1093/brain/awn065
Abstract
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.This publication has 39 references indexed in Scilit:
- Patterns of levodopa response in Parkinson's disease: a clinico-pathological studyBrain, 2007
- Causes of death in multiple system atrophyJournal of Neurology, Neurosurgery & Psychiatry, 2006
- Predictors of falls and fractures in bradykinetic rigid syndromes: a retrospective studyJournal of Neurology, Neurosurgery & Psychiatry, 2006
- Natural History of Progressive Supranuclear Palsy: A Clinicopathologic Study from a Population of Brain DonorsEuropean Neurology, 2005
- Toward future therapies in progressive supranuclear palsyMovement Disorders, 2005
- The spectrum of pathological involvement of the striatonigral and olivopontocerebellar systems in multiple system atrophy: clinicopathological correlationsBrain, 2004
- Comparison of natural histories of progressive supranuclear palsy and multiple system atrophyNeurological Sciences, 2001
- Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study.Journal of Neurology, Neurosurgery & Psychiatry, 1996
- ?Minimal change? multiple system atrophyMovement Disorders, 1994
- Idiopathic Parkinson's disease combined with multiple system atrophy. A clinicopathological reportMovement Disorders, 1991