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Journal of Neural Transmission

, Volume 112, Issue 12, pp 1677–1686 | Cite as

The European Multiple System Atrophy-Study Group (EMSA-SG)

  • F. Geser
  • K. Seppi
  • M. Stampfer-Kountchev
  • M. Köllensperger
  • A. Diem
  • J. P. Ndayisaba
  • K. Ostergaard
  • E. Dupont
  • A. Cardozo
  • E. Tolosa
  • M. Abele
  • R. Dodel
  • T. Klockgether
  • I. Ghorayeb
  • F. Yekhlef
  • F. Tison
  • C. Daniels
  • F. Kopper
  • G. Deuschl
  • M. Coelho
  • J. Ferreira
  • M. M. Rosa
  • C. Sampaio
  • M. Bozi
  • A. Schrag
  • J. Hooker
  • H. Kim
  • T. Scaravilli
  • C. J. Mathias
  • C. Fowler
  • N. Wood
  • N. Quinn
  • H. Widner
  • C. F. Nilsson
  • O. Lindvall
  • N. Schimke
  • K. M. Eggert
  • W. Oertel
  • F. del Sorbo
  • F. Carella
  • A. Albanese
  • M. T. Pellecchia
  • P. Barone
  • R. Djaldetti
  • G. Meco
  • C. Colosimo
  • A. Gonzalez-Mandly
  • J. Berciano
  • T. Gurevich
  • N. Giladi
  • M. Galitzky
  • F. Ory
  • O. Rascol
  • C. Kamm
  • K. Buerk
  • S. Maaß
  • T. Gasser
  • W. Poewe
  • G. K. Wenning
  • on behalf of the EMSA-SG
Article

Summary.

Introduction. The European Multiple System Atrophy-Study Group (EMSA-SG) is an academic network comprising 23 centers across Europe and Israel that has constituted itself already in January 1999. This international forum of established experts under the guidance of the University Hospital of Innsbruck as coordinating center is supported by the 5th framework program of the European Union since March 2001 (QLK6-CT-2000-00661). Objectives. Primary goals of the network include (1) a central Registry for European multiple system atrophy (MSA) patients, (2) a decentralized DNA Bank, (3) the development and validation of the novel Unified MSA Rating Scale (UMSARS), (4) the conduction of a Natural History Study (NHS), and (5) the planning or implementation of interventional therapeutic trials. Methods. The EMSA-SG Registry is a computerized data bank localized at the coordinating centre in Innsbruck collecting diagnostic and therapeutic data of MSA patients. Blood samples of patients and controls are recruited into the DNA Bank. The UMSARS is a novel specific rating instrument that has been developed and validated by the EMSA-SG. The NHS comprises assessments of basic anthropometric data as well as a range of scales including the UMSARS, Unified Parkinson’s Disease Rating Scale (UPDRS), measures of global disability, Red Flag list, MMSE (Mini Mental State Examination), quality of live measures, i.e. EuroQoL 5D (EQ-5D) and Medical Outcome Study Short Form (SF-36) as well as the Beck Depression Inventory (BDI). In a subgroup of patients dysautonomic features are recorded in detail using the Queen Square Cardiovascular Autonomic Function Test Battery, the Composite Autonomic Symptom Scale (COMPASS) and measurements of residual urinary volume. Most of these measures are repeated at 6-monthly follow up visits for a total study period of 24 months. Surrogate markers of the disease progression are identified by the EMSA-SG using magnetic resonance and diffusion weighted imaging (MRI and DWI, respectively). Results. 412 patients have been recruited into the Registry so far. Probable MSA-P was the most common diagnosis (49% of cases). 507 patients donated DNA for research. 131 patients have been recruited into the NHS. There was a rapid deterioration of the motor disorder (in particular akinesia) by 26.1% of the UMSARS II, and – to a lesser degree – of activities of daily living by 16.8% of the UMSARS I in relation to the respective baseline scores. Motor progression was associated with low motor or global disability as well as low akinesia or cerebellar subscores at baseline. Mental function did not deteriorate during this short follow up period. Conclusion. For the first time, prospective data concerning disease progression are available. Such data about the natural history and prognosis of MSA as well as surrogate markers of disease process allow planning and implementation of multi-centre phase II/III neuroprotective intervention trials within the next years more effectively. Indeed, a trial on growth hormone in MSA has just been completed, and another on minocycline will be completed by the end of this year.

Keywords: Multiple system atrophy, European Multiple System Atrophy-Study Group, Registry, DNA Bank, Unified MSA Rating Scale, Natural History Study, therapeutic trials. 

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References

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Copyright information

© Springer-Verlag/Wien 2005

Authors and Affiliations

  • F. Geser
    • 1
  • K. Seppi
    • 1
  • M. Stampfer-Kountchev
    • 1
  • M. Köllensperger
    • 1
  • A. Diem
    • 1
  • J. P. Ndayisaba
    • 1
  • K. Ostergaard
    • 2
  • E. Dupont
    • 2
  • A. Cardozo
    • 3
  • E. Tolosa
    • 3
  • M. Abele
    • 4
  • R. Dodel
    • 4
  • T. Klockgether
    • 4
  • I. Ghorayeb
    • 5
  • F. Yekhlef
    • 5
  • F. Tison
    • 5
  • C. Daniels
    • 6
  • F. Kopper
    • 6
  • G. Deuschl
    • 6
  • M. Coelho
    • 7
  • J. Ferreira
    • 7
  • M. M. Rosa
    • 7
  • C. Sampaio
    • 7
  • M. Bozi
    • 8
  • A. Schrag
    • 9
  • J. Hooker
    • 8
  • H. Kim
    • 8
  • T. Scaravilli
    • 8
  • C. J. Mathias
    • 10
  • C. Fowler
    • 11
  • N. Wood
    • 12
  • N. Quinn
    • 8
  • H. Widner
    • 13
  • C. F. Nilsson
    • 13
  • O. Lindvall
    • 13
  • N. Schimke
    • 14
  • K. M. Eggert
    • 14
  • W. Oertel
    • 14
  • F. del Sorbo
    • 15
  • F. Carella
    • 15
  • A. Albanese
    • 15
  • M. T. Pellecchia
    • 16
  • P. Barone
    • 16
  • R. Djaldetti
    • 17
  • G. Meco
    • 18
  • C. Colosimo
    • 18
  • A. Gonzalez-Mandly
    • 19
  • J. Berciano
    • 19
  • T. Gurevich
    • 20
  • N. Giladi
    • 20
  • M. Galitzky
    • 21
  • F. Ory
    • 21
  • O. Rascol
    • 21
  • C. Kamm
    • 22
  • K. Buerk
    • 22
  • S. Maaß
    • 23
  • T. Gasser
    • 22
  • W. Poewe
    • 1
  • G. K. Wenning
    • 1
  • on behalf of the EMSA-SG
  1. 1.Clinical Department of NeurologyInnsbruck Medical UniversityAustria
  2. 2.Department of NeurologyAarhus University HospitalAarhusDenmark
  3. 3.Department of NeurologyUniversitat de Barcelona, Hospital ClínicSpain
  4. 4.Department of NeurologyUniversity of BonnGermany
  5. 5.Service de Neurologie, Hopital du Haut-LevequePessacFrance
  6. 6.Department of NeurologyChristian-Albrechts University of KielGermany
  7. 7.Faculdade de Medicina de Lisboa, Hopital Santa Maria, Centro de Neurosciencias de LisboaPortugal
  8. 8.University College London, Institute of NeurologyQueen SquareUnited Kingdom
  9. 9.Department of Clinical NeurosciencesRoyal Free and University College Medical SchoolUnited Kingdom
  10. 10.Neurovascular Medicine UnitImperial College London at St Mary’s HospitalUnited Kingdom
  11. 11.Department of Uro-NeurologyNational Hospital for Neurology and NeurosurgeryUnited Kingdom
  12. 12.Department of Molecular NeuroscienceInstitute of Neurology, University College LondonUnited Kingdom
  13. 13.Department of Clinical Neuroscience, Division of NeurologyUniversity of LundSweden
  14. 14.Department of NeurologyPhilipps-University MarburgGermany
  15. 15.Istituto Carlo BestaMilanoItaly
  16. 16.Department of Neurological SciencesUniversity Federico IINaplesItaly
  17. 17.Department of NeurologyRabin Medical CenterPetach-TiqvaIsrael
  18. 18.Department of Neurological SciencesLa Sapienza UniversityRomeItaly
  19. 19.Service of Neurology, University Hospital Marques de ValdecillaSantanderSpain
  20. 20.Movement Disorders Unit, Department of NeurologyTel Aviv Sourasky Medical CenterIsrael
  21. 21.Laboratoire de Pharmacologie, Faculte de MedecineToulouse III UniversityToulouseFrance
  22. 22.Department of Neurodegenerative DiseasesHertie-Institute for Clinical Brain Research, University of TuebingenGermany
  23. 23.Department of NeurologyLudwig-Maximilians-University MunichGermany

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