This is a very interesting article written by some of Europe’s top PSP researchers (and presumably top MSA researchers too). The first author is O’Sullivan. This is also a very important paper because it includes analysis of brains donated to the University College of London brain bank.
110 pathologically-confirmed PSP cases and 83 pathologically-confirmed MSA cases were examined for early clinical features and survival. PSP cases were divided according to the D. Williams criteria of Richardson’s syndrome (RS) and PSP-parkinsonism. MSA cases were divided according to the presence of early autonomic failure.
The PSP findings confirms what D. Williams has told us before:
“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.”
The MSA findings are new to me (though maybe not to many of you):
“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.”
Interesting (and scary?) that “not being admitted to residential care” predicts *shorter* disease duration in MSA.
I’ve copied below the abstract.
Update: the full paper is now available at no charge. See our post here with links.
Robin
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Brain. 2008 Apr 2 [Epub ahead of print]
Clinical outcomes of progressive supranuclear palsy and multiple system atrophy.
O’Sullivan SS, Massey LA, Williams DR, Silveira-Moriyama L, Kempster PA, Holton JL, Revesz T, Lees AJ.
Reta Lila Weston Institute of Neurological Studies, Institute of Neurology, Queen Square Brain Bank for Neurological Disorders and Institute of Neurology, Sara Koe PSP Research Centre, Institute of Neurology, University College London, London, UK, Faculty of Medicine (Neuroscience), Monash University (Alfred Hospital Campus) and Department of Neurosciences, Monash Medical Centre, Melbourne, Australia.
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.
PMID: 18385183