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Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry.
Mov Disord. 2010 Nov 15; 25(15):2604-12.MD

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.

Authors+Show Affiliations

Clinical Department of Neurology, Innsbruck Medical University, Austria.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Multicenter Study
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

20922810

Citation

Köllensperger, Martin, et al. "Presentation, Diagnosis, and Management of Multiple System Atrophy in Europe: Final Analysis of the European Multiple System Atrophy Registry." Movement Disorders : Official Journal of the Movement Disorder Society, vol. 25, no. 15, 2010, pp. 2604-12.
Köllensperger M, Geser F, Ndayisaba JP, et al. Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry. Mov Disord. 2010;25(15):2604-12.
Köllensperger, M., Geser, F., Ndayisaba, J. P., Boesch, S., Seppi, K., Ostergaard, K., Dupont, E., Cardozo, A., Tolosa, E., Abele, M., Klockgether, T., Yekhlef, F., Tison, F., Daniels, C., Deuschl, G., Coelho, M., Sampaio, C., Bozi, M., Quinn, N., ... Wenning, G. K. (2010). Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry. Movement Disorders : Official Journal of the Movement Disorder Society, 25(15), 2604-12. https://doi.org/10.1002/mds.23192
Köllensperger M, et al. Presentation, Diagnosis, and Management of Multiple System Atrophy in Europe: Final Analysis of the European Multiple System Atrophy Registry. Mov Disord. 2010 Nov 15;25(15):2604-12. PubMed PMID: 20922810.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Presentation, diagnosis, and management of multiple system atrophy in Europe: final analysis of the European multiple system atrophy registry. AU - Köllensperger,Martin, AU - Geser,Felix, AU - Ndayisaba,Jean-Pierre, AU - Boesch,Sylvia, AU - Seppi,Klaus, AU - Ostergaard,Karen, AU - Dupont,Erik, AU - Cardozo,A, AU - Tolosa,Eduardo, AU - Abele,Michael, AU - Klockgether,Thomas, AU - Yekhlef,Farid, AU - Tison,Francois, AU - Daniels,Christine, AU - Deuschl,Günther, AU - Coelho,Miguel, AU - Sampaio,Cristina, AU - Bozi,Maria, AU - Quinn,Niall, AU - Schrag,Anette, AU - Mathias,Chris J, AU - Fowler,Clare, AU - Nilsson,Christer F, AU - Widner,Håkan, AU - Schimke,Nicole, AU - Oertel,Wolfgang, AU - Del Sorbo,Francesca, AU - Albanese,Alberto, AU - Pellecchia,Maria Teresa, AU - Barone,Paolo, AU - Djaldetti,Ruth, AU - Colosimo,Carlo, AU - Meco,Giuseppe, AU - Gonzalez-Mandly,Antonio, AU - Berciano,Jose, AU - Gurevich,Tanya, AU - Giladi,Nir, AU - Galitzky,Monique, AU - Rascol,Olivier, AU - Kamm,Christoph, AU - Gasser,Thomas, AU - Siebert,Uwe, AU - Poewe,Werner, AU - Wenning,Gregor K, AU - ,, PY - 2009/01/11/received PY - 2009/04/27/revised PY - 2010/03/22/accepted PY - 2010/10/6/entrez PY - 2010/10/6/pubmed PY - 2011/2/26/medline SP - 2604 EP - 12 JF - Movement disorders : official journal of the Movement Disorder Society JO - Mov Disord VL - 25 IS - 15 N2 - 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. SN - 1531-8257 UR - https://www.unboundmedicine.com/medline/citation/20922810/Presentation_diagnosis_and_management_of_multiple_system_atrophy_in_Europe:_final_analysis_of_the_European_multiple_system_atrophy_registry_ L2 - https://doi.org/10.1002/mds.23192 DB - PRIME DP - Unbound Medicine ER -