Progression of parkinsonism in multiple system atrophy

Deutsche Zeitschrift für Nervenheilkunde - Tập 252 - Trang 91-96 - 2005
Klaus Seppi1, Farid Yekhlef2, Anja Diem1, Elisabeth Luginger Wolf1, Joerg Mueller1, François Tison3, Niall P. Quinn4, Werner Poewe1, Gregor K. Wenning1
1Department of Neurology, University Hospital, Innsbruck, Austria
2Fédération de Neurologie Epidémiologie et Biostatistiques, Centre Hospitalo-Universitaire de Bordeaux, Bordeaux, France
3Institut National de la Santé et de la Recherche Médicale U-330, Epidémiologie et Biostatistiques, Centre Hospitalo-Universitaire de Bordeaux, Bordeaux, France
4Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology, London, UK

Tóm tắt

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

Tài liệu tham khảo

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