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Presentation, diagnosis, and management of multiple system atrophy in Europe: Final analysis of the European multiple system atrophy registry
Author(s) -
Köllensperger Martin,
Geser Felix,
Ndayisaba JeanPierre,
Boesch Sylvia,
Seppi Klaus,
Ostergaard Karen,
Dupont Erik,
Cardozo A.,
Tolosa Eduardo,
Abele Michael,
Klockgether Thomas,
Yekhlef Farid,
Tison Francois,
Daniels Christine,
Deuschl Günther,
Coelho Miguel,
Sampaio Cristina,
Bozi Maria,
Quinn Niall,
Schrag Anette,
Mathias Chris J.,
Fowler Clare,
Nilsson Christer F.,
Widner Håkan,
Schimke Nicole,
Oertel Wolfgang,
del Sorbo Francesca,
Albanese Alberto,
Pellecchia Maria Teresa,
Barone Paolo,
Djaldetti Ruth,
Colosimo Carlo,
Meco Giuseppe,
GonzalezMandly Antonio,
Berciano Jose,
Gurevich Tanya,
Giladi Nir,
Galitzky Monique,
Rascol Olivier,
Kamm Christoph,
Gasser Thomas,
Siebert Uwe,
Poewe Werner,
Wenning Gregor K.
Publication year - 2010
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.23192
Subject(s) - dysautonomia , parkinsonism , medicine , atrophy , cerebellar ataxia , orthostatic vital signs , olivopontocerebellar atrophy , progressive supranuclear palsy , pediatrics , ataxia , parkinson's disease , neuroradiology , disease , neurology , degenerative disease , psychiatry , blood pressure
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. © 2010 Movement Disorder Society