Abstract
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.
Conclusion
This is the first observational study on UPDRS rates of decline in MSA. The observed 28.6% annual increase of UPDRSIII 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.
Article PDF
Similar content being viewed by others
References
Colosimo C, Merello M, Pontieri FE (1996) Amantadine in parkinsonian patients unresponsive to levodopa: a pilot study. J Neurol 243:422–425
Fahn S, Elton Rl, Members of the UPDRS Development Committee (1987) Unified Parkinson’s disease Rating Scale. In: Fahn S, Marsden CD, Calne DB, Goldstein M (eds) Recent developments in Parkinson’s disease, Vol 2. Florham Park, NJ: MacMillan Healthcare Information; pp 153–163:293–304, NJ
Fetoni V, Soliveri P, Monza D, Testa D, Girotti F (1999) Affective symptoms in multiple system atrophy and Parkinson’s disease: response to levodopa therapy. J Neurol Neurosurg Psychiatry 66:541–544
Gilman S, Low PA, Quinn N, Albanese A, Ben-Shlomo Y, Fowler CJ, Kaufmann H, Klockgether T, Lang AE, Lantos PL, Litvan I, Mathias CJ, Oliver E, Robertson D, Schatz I, Wenning GK (1999) Consensus statement on the diagnosis of multiple system atrophy (see comments). J Neurol Sci 163:94–98
Goetz CG, Stebbins GT, Blasucci LM (2000) Differential progression of motor impairment in levodopa-treated Parkinson’s disease. Mov Disord 15:479–484
Hughes AJ, Daniel SE, Kilford L, Lees AJ (1992) Accuracy of clinical diagnosis of idiopathic Parkinson’s disease: a clinico-pathological study of 100 cases (see comments). J Neurol Neurosurg Psychiatry 55:181–184
Jankovic J, Kapadia AS (2001) Functional decline in Parkinson disease. Arch Neurol 58:1611–161
Lantos PL (1998) The definition of multiple system atrophy: a review of recent developments. J Neuropathol Exp Neurol 57:1099–1111
Lee CS, Schulzer M, Mak EK, Snow BJ, Tsui JK, Calne S, Hammerstad J, Calne DB (1994) Clinical observations on the rate of progression of idiopathic parkinsonism. Brain 117 (Pt 3):501–507
Litvan I, Goetz CG, Jankovic J, Wenning GK, Booth V, Bartko JJ, McKee A, Jellinger K, Lai EC, Brandel JP, Verny M, Chaudhuri KR, Pearce RK, Agid Y (1997) What is the accuracy of the clinical diagnosis of multiple system atrophy? A clinicopathologic study. Arch Neurol 54:937–944
Louis ED, Tang MX, Cote L, Alfaro B, Mejia H, Marder K (1999) Progression of parkinsonian signs in Parkinson disease. Arch Neurol 56:334–337
Muller J, Wenning GK, Jellinger K, Mc-Kee A, Poewe W, Litvan I (2000) Progression of Hoehn and Yahr stages in Parkinsonian disorders: a clinicopathologic study. Neurology 55:888–891
Olanow CW, Hauser RA, Gauger L, Malapira T, Koller W, Hubble J, Bushenbark K, Lilienfeld D, Esterlitz J (1995) The effect of deprenyl and levodopa on the progression of Parkinson’s disease. Ann Neurol 38:771–777
Ostergaard K, Sunde N, Dupont E (2002) Effects of bilateral stimulation of the subthalamic nucleus in patients with severe Parkinson’s disease and motor fluctuations. Mov Disord 17:693–700
Poewe WH, Wenning GK (1998) The natural history of Parkinson’s disease. Ann Neurol 44:S1–S9
Quinn NP, Marsden CD (1993) The motor disorder of multiple system atrophy. J Neurol Neurosurg Psychiatry 56:1239–1242
Rossi P, Colosimo C, Moro E, Tonali P, Albanese A (2000) Acute challenge with apomorphine and levodopa in Parkinsonism. Eur Neurol 43:95–101
Saito Y, Matsuoka Y, Takahashi A, Ohno Y (1994) Survival of patients with multiple system atrophy. Intern Med 33:321–325
Schulz JB, Klockgether T, Petersen D, Jauch M, Muller-Schauenburg W, Spieker S, Voigt K, Dichgans J (1994) Multiple system atrophy: natural history, MRI morphology, and dopamine receptor imaging with 123IBZM-SPECT. J Neurol Neurosurg Psychiatry 57:1047–1056
Testa D, Filippini G, Farinotti M, Palazzini E, Caraceni T (1996) Survival in multiple system atrophy: a study of prognostic factors in 59 cases. J Neurol 243:401–404
Tison F, Yekhlef F, Chrysostome V, Balestre E, Quinn NP, Poewe W, Wenning GK (2002) Parkinsonism in multiple system atrophy: natural history, severity (UPDRS-III), and disability assessment compared with Parkinson’s disease. Mov Disord 17:701–709
Watanabe H, Saito Y, Terao S, Ando T, Kachi T, Mukai E,Aiba I, Abe Y, Tamakoshi A, Doyu M, Hirayama M, Sobue G (2002) Progression and prognosis in multiple system atrophy: An analysis of 230 Japanese patients. Brain 125:1070–1083
Wenning GK, Ben Shlomo Y, Magalhaes M, Daniel SE, Quinn NP (1994) Clinical features and natural history of multiple system atrophy.An analysis of 100 cases. Brain 117(Pt 4):835–845
Wenning GK, Quinn N, Magalhaes M, Mathias C, Daniel SE (1994) “Minimal change” multiple system atrophy. Mov Disord 9:161–166
Wenning GK, Quinn NP (1997) Parkinsonism. Multiple system atrophy. Baillieres Clin Neurol 6:187–204
Wenning GK, Seppi K, Sampaio C, Quinn NP, Poewe W, Tison F (2002) European Multiple System Atrophy Study Group (EMSA-SG): Validation of the Unified Multiple System Atrophy Rating Scale (UMSARS) (Abstract). Mov Disord 17:S252
Wenning GK, Tison F, Ben-Shlomo Y, Daniel SE, Quinn NP (1997) Multiple system atrophy: a review of 203 pathologically proven cases. Mov Disord 12:133–147
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Seppi, K., Yekhlef, F., Diem, A. et al. Progression of parkinsonism in multiple system atrophy. J Neurol 252, 91–96 (2005). https://doi.org/10.1007/s00415-005-0617-2
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/s00415-005-0617-2