Presentation, diagnosis, and management of multiple system atrophy in Europe: Final analysis of the European multiple system atrophy registry

Martin Köllensperger, Felix Geser, Jean Pierre Ndayisaba, Sylvia Boesch, Klaus Seppi, Karen Ostergaard, Erik Dupont, A. Cardozo, Eduardo Tolosa, Michael Abele, Thomas Klockgether, Farid Yekhlef, Francois Tison, Christine Daniels, Günther Deuschl, Miguel Coelho, Cristina Sampaio, Maria Bozi, Niall Quinn, Anette SchragChris J. Mathias, Clare Fowler, Christer F. Nilsson, Håkan Widner, Nicole Schimke, Wolfgang Oertel, Francesca Del Sorbo, Alberto Albanese, Maria Teresa Pellecchia, Paolo Barone, Ruth Djaldetti, Carlo Colosimo, Giuseppe Meco, Antonio Gonzalez-Mandly, Jose Berciano, Tanya Gurevich, Nir Giladi, Monique Galitzky, Olivier Rascol, Christoph Kamm, Thomas Gasser, Uwe Siebert, Werner Poewe, Gregor K. Wenning

Research output: Contribution to journalArticlepeer-review

Abstract

Multiple system atrophy (MSA) is a Parkinson's Disease (PD)-like α-synucleinopathy clinically characterized by dysautonomia, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination. We aimed to determine whether the clinical presentation of MSA as well as diagnostic and therapeutic strategies differ across Europe and Israel. In 19 European MSA Study Group centres all consecutive patients with a clinical diagnosis of MSA were recruited from 2001 to 2005. A standardized minimal data set was obtained from all patients. Four-hundred thirty-seven MSA patients from 19 centres in 10 countries were included. Mean age at onset was 57.8 years; mean disease duration at inclusion was 5.8 years. According to the consensus criteria 68% were classified as parkinsonian type (MSA-P) and 32% as cerebellar type (MSA-C) (probable MSA: 72%, possible MSA: 28%). Symptomatic dysautonomia was present in almost all patients, and urinary dysfunction (83%) more common than symptomatic orthostatic hypotension (75%). Cerebellar ataxia was present in 64%, and parkinsonism in 87%, of all cases. No significant differences in the clinical presentation were observed between the participating countries. In contrast, diagnostic work up and therapeutic strategies were heterogeneous. Less than a third of patients with documented orthostatic hypotension or neurogenic bladder disturbance were receiving treatment. This largest clinical series of MSA patients reported so far shows that the disease presents uniformly across Europe. The observed differences in diagnostic and therapeutic management including lack of therapy for dysautonomia emphasize the need for future guidelines in these areas.

Original languageEnglish
Pages (from-to)2604-2612
Number of pages9
JournalMovement Disorders
Volume25
Issue number15
DOIs
Publication statusPublished - Nov 2010

Keywords

  • EMSA-SG
  • Multiple system atrophy
  • Natural history

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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