Advanced

Presentation, Diagnosis, and Management of Multiple System Atrophy in Europe: Final Analysis of the European Multiple System Atrophy Registry

Koellensperger, Martin; Geser, Felix; Ndayisaba, Jean-Pierre; Boesch, Sylvia; Seppi, Klaus; Ostergaard, Karen; Dupont, Erik; Cardozo, A.; Tolosa, Eduardo and Abele, Michael, et al. (2010) In Movement Disorders 25(15). p.2604-2612
Abstract
Multiple system atrophy (MSA) is a Parkinson's Disease (PD)-like alpha-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... (More)
Multiple system atrophy (MSA) is a Parkinson's Disease (PD)-like alpha-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. (C) 2010 Movement Disorder Society (Less)
Please use this url to cite or link to this publication:
author
, et al. (More)
(Less)
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
EMSA-SG, multiple system atrophy, natural history
in
Movement Disorders
volume
25
issue
15
pages
2604 - 2612
publisher
John Wiley & Sons
external identifiers
  • wos:000284060600018
  • scopus:78349241725
ISSN
0885-3185
DOI
10.1002/mds.23192
language
English
LU publication?
yes
id
0e00414f-3dac-41f6-aa92-050d9f930940 (old id 1753051)
date added to LUP
2011-01-04 08:04:20
date last changed
2018-07-01 03:09:20
@article{0e00414f-3dac-41f6-aa92-050d9f930940,
  abstract     = {Multiple system atrophy (MSA) is a Parkinson's Disease (PD)-like alpha-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. (C) 2010 Movement Disorder Society},
  author       = {Koellensperger, Martin and Geser, Felix and Ndayisaba, Jean-Pierre and Boesch, Sylvia and Seppi, Klaus and Ostergaard, Karen and Dupont, Erik and Cardozo, A. and Tolosa, Eduardo and Abele, Michael and Klockgether, Thomas and Yekhlef, Farid and Tison, Francois and Daniels, Christine and Deuschl, Guenther and Coelho, Miguel and Sampaio, Cristina and Bozi, Maria and Quinn, Niall and Schrag, Anette and Mathias, Chris J. and Fowler, Clare and Nilsson, Christer and Widner, Håkan and Schimke, Nicole and Oertel, Wolfgang and del Sorbo, Francesca and Albanese, Alberto and Pellecchia, Maria Teresa and Barone, Paolo and Djaldetti, Ruth and Colosimo, Carlo and Meco, Giuseppe and Gonzalez-Mandly, Antonio and Berciano, Jose and Gurevich, Tanya and Giladi, Nir and Galitzky, Monique and Rascol, Olivier and Kamm, Christoph and Gasser, Thomas and Siebert, Uwe and Poewe, Werner and Wenning, Gregor K.},
  issn         = {0885-3185},
  keyword      = {EMSA-SG,multiple system atrophy,natural history},
  language     = {eng},
  number       = {15},
  pages        = {2604--2612},
  publisher    = {John Wiley & Sons},
  series       = {Movement Disorders},
  title        = {Presentation, Diagnosis, and Management of Multiple System Atrophy in Europe: Final Analysis of the European Multiple System Atrophy Registry},
  url          = {http://dx.doi.org/10.1002/mds.23192},
  volume       = {25},
  year         = {2010},
}