Brain Matters

 

Very Important Article

This is a very interesting and important article written by some of Europe's top PSP researchers (and presumably top MSA researchers too).  

A large number -- 110 PSP cases and 83 MSA cases -- of pathologically-confirmed cases were examined for early clinical features and survival.  PSP cases were divided according to the criteria of Richardson's syndrome (RS) and PSP-parkinsonism.  MSA cases were divided according to the presence of early autonomic failure.

Key Findings

The PSP findings confirm what one of the authors 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 were:  "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."

Here's the abstract for this important article:  (we've added the paragraph breaks)

"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."

Age of Onset, Time Between Onset and Diagnosis, Disease Duration, Accuracy of Clinical Diagnosis

This chart provides key data for PSP and MSA, by subtype -- age of onset, time between onset and diagnosis, disease duration, age at death, and accuracy of clinical diagnosis. 
http://brain.oxfordjournals.org/cgi/content-nw/full/131/5/1362/T1

The same chart shows the extent to which research participants were responsive to L-dopa, and whether the response was sustained.

Survival Time, Based on Milestones

This chart shows the survival time for PSP and MSA, by subtype, after reaching various milestones, including frequent falls, wheelchair dependance, unintelligible speech, severe dysphagia, urinary catheter, cognitive impairment, residential care, and first milestone:
http://brain.oxfordjournals.org/cgi/content-nw/full/131/5/1362/T3

Full Article

You can read the full article online for free from this link on the journal Brain's website:

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