Progression of parkinsonism in multiple system atrophy.
BACKGROUND
Progression of parkinsonian motor impairment is usually rapid and relentless in multiple system atrophy (MSA). However, it may also be subject to considerable variation. Prospective natural history studies using validated rating scales are required to accurately determine the progression of parkinsonism in MSA.


OBJECTIVE
To assess the progression of parkinsonism in patients with the Parkinson variant of MSA.


METHODS
Parkinsonian motor impairment was assessed on regular therapy at two time points (mean follow-up 11.8 months, SD 1.4) using the Hoehn and Yahr scale (H&Y), the Schwab and England ADL scale (SES) and the motor examination section of the UPDRS (UPDRS-III) in 38 patients with clinically probable MSA-P.


RESULTS
We examined 38 patients with probable MSA-P (mean age 63.2 years, SD 7.4; mean disease duration 4.1 years, SD 3.0). The mean difference of UPDRS-III between baseline and follow-up was 10.8 (95% CI 8.6-12.9), consistent with an average annual 28.3 % increase of UPDRS-III baseline scores. Several variables were associated with faster progression of parkinsonism including low baseline global motor disability as assessed by H&Y and SES, low baseline UPDRS-III score, and short disease duration. UPDRS-III progression was unrelated to gender, age at symptom onset, and age at baseline visit.


CONCLUSIONS
This is the first observational study on UPDRS rates of decline in MSA. The observed 28.6% annual increase of UPDRS-III scores illustrates the rapid progression of motor impairment in MSA. Furthermore, motor progression appeared to be accelerated during the early disease stages. Our data allow sample size calculations that may be helpful for the planning of future therapeutic trials.
Seppi
Klaus
K
Department of Neurology, University Hospital, Innsbruck, Austria.
Yekhlef
Farid
F
Diem
Anja
A
Luginger Wolf
Elisabeth
E
Mueller
Joerg
J
Tison
François
F
Quinn
Niall P
NP
Poewe
Werner
W
Wenning
Gregor K
GK
D017668:Age of Onset; D000368:Aged; D004185:Disability Evaluation; D018450:Disease Progression; D018491:Dopamine Agonists / Q000627:therapeutic use; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000150:complications / Q000503:physiopathology*; D009460:Neurologic Examination / Q000592:standards; D020734:Parkinsonian Disorders / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology*; D011446:Prospective Studies
D018491:Dopamine Agonists