Progression of multiple system atrophy (MSA): A prospective natural history study by the European MSA study group (EMSA SG)

Felix Geser, Gregor K. Wenning, Klaus Seppi, Michaela Stampfer-Kountchev, Christoph Scherfler, Martin Sawires, Carolin Frick, Jean Pierre Ndayisaba, Hanno Ulmer, Maria T. Pellecchia, Paolo Barone, Hee T. Kim, Juzar Hooker, Niall P. Quinn, Adriana Cardozo, Eduardo Tolosa, Michael Abele, Thomas Klockgether, Karen Østergaard, Erik DupontNicole Schimke, Karla M. Eggert, Wolfgang Oertel, Ruth Djaldetti, Werner Poewe, Richard Dodel, François Tison, Imad Ghorayeb, Pierre Pollak, Martin Kölensperger, Anja Diem, Günther Deuschl, Christine Daniels, Florian Kopper, Cristina Sampaio, Miguel Coelho, Joaquim Ferreira, Mário M. Rosa, Zvezdan Pirtosek, Andrew J. Lees, David J. Brooks, Clare Fowler, Tamas Revesz, Alexander Gerhard, Janice Holton, Anette Schrag, Nick Wood, Maria Bozi, Tomaso Scaravilli, Olle Lindvall, Håkan Widner, Christer F. Nilsson, Martin Grabowski, Andrea Krisp, Corsten Möller, Alberto Albanese, Francesca del Sorbo, Francesco Carella, Giuseppe Meco, Carlo Colosimo, Jose Berciano, Andres Gonzalez-Mandly, Nir Giladi, Tanya Gurevich, Olivier Rascol, Monique Galitzky, Fabienne Ory, Thomas Gasser, Christoph Kamm, K. Buerk, Sylvia Maass, Sten Magnus Aquilonius, Jonas Bergquist

Research output: Contribution to journalArticlepeer-review

Abstract

The disease-specific Unified Multiple System Atrophy Rating Scale (UMSARS) has been developed recently and validated for assessing disease severity in multiple system atrophy (MSA). Here, we aimed at (1) assessing rates of disease progression in MSA and (2) validating UMSARS for sensitivity to change over time. Impairment was assessed at two time points 12 months apart using UMSARS Part I (historical review), UMSARS Part II (motor examination), as well as measures of global disease severity, including UMSARS Part IV, Hoehn and Yahr (HY) Parkinson's disease staging, Schwab England Activities of Daily Living (SE ADL), and a three-point global Severity Scale (SS3). Fifty patients (male:female ratio, 1:0.9; possible MSA, 16%; probable MSA, 84%; MSA-parkinsonian, 58%; MSA-cerebellar, 42%) were assessed twice with an interval of 12.3 months. UMSARS II scores progressed by 57.3% (P <0.0001) and UMSARS I scores by 35.6% (P <0.0001) in relation to the respective baseline scores with no differences between motor subtypes, diagnostic categories and gender. Significant inverse correlations between (1) UMSARS I or UMSARS II progression and (2) baseline disability measures (i.e., the respective UMSARS or SS3 scores) and disease duration were found. Furthermore, the increases in HY staging, SE ADL and SS3 correlated significantly with UMSARS I, UMSARS II, and UMSARS IV progression. This report is the first prospective study showing rapid annual UMSARS rates of decline in MSA. Our data contribute to the ongoing validation process of UMSARS, and they facilitate the planning and implementation of future neuroprotective intervention trials.

Original languageEnglish
Pages (from-to)179-186
Number of pages8
JournalMovement Disorders
Volume21
Issue number2
DOIs
Publication statusPublished - Feb 2006

Keywords

  • Multiple system atrophy
  • Progression
  • Sensitivity to change
  • Unified MSA Rating Scale

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Fingerprint Dive into the research topics of 'Progression of multiple system atrophy (MSA): A prospective natural history study by the European MSA study group (EMSA SG)'. Together they form a unique fingerprint.

Cite this