Document Detail

Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry.
MedLine Citation:
PMID:  20922810     Owner:  NLM     Status:  In-Process    
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.
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 Schrag; Chris 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;
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Movement disorders : official journal of the Movement Disorder Society     Volume:  25     ISSN:  1531-8257     ISO Abbreviation:  Mov. Disord.     Publication Date:  2010 Nov 
Date Detail:
Created Date:  2010-11-11     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8610688     Medline TA:  Mov Disord     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2604-12     Citation Subset:  IM    
Copyright Information:
© 2010 Movement Disorder Society.
Clinical Department of Neurology, Innsbruck Medical University, Austria.
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