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

Geser, F; Seppi, K; Stampfer-Kountchev, M; Kollensperger, M; Diem, A; Ndayisaba, JP; Ostergaard, K; Dupont, E; Cardozo, A and Tolosa, E, et al. (2005) In Journal of Neural Transmission 112(12). p.1677-1686
Abstract
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... (More)
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. (Less)
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Natural History Study, Unified MSA Rating Scale, DNA Bank, Registry, multiple system atrophy, European Multiple System Atrophy-Study Group, therapeutic trials
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Journal of Neural Transmission
volume
112
issue
12
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1677 - 1686
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Springer
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  • pmid:16049636
  • wos:000233262700009
  • scopus:27744545323
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0300-9564
DOI
10.1007/s00702-005-0328-y
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English
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@article{7c08e415-eda9-4f3f-9273-5198116d3609,
  abstract     = {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.},
  author       = {Geser, F and Seppi, K and Stampfer-Kountchev, M and Kollensperger, M and Diem, A and Ndayisaba, JP and Ostergaard, K and Dupont, E and Cardozo, A and Tolosa, E and Abele, M and Dodel, R and Klockgether, T and Ghorayeb, I and Yekhlef, F and Tison, F and Daniels, C and Kopper, F and Deuschl, G and Coelho, M and Ferreira, J and Rosa, MM and Sampaio, C and Bozi, M and Schrag, A and Hooker, J and Kim, H and Scaravilli, T and Mathias, CJ and Fowler, C and Wood, N and Quinn, N and Widner, Håkan and Nilsson, CF and Lindvall, Olle and Schimke, N and Eggert, KM and Oertel, W and del Sorbo, F and Carella, F and Albanese, A and Pellecchia, MT and Barone, P and Djaldetti, R and Meco, G and Colosimo, C and Gonzalez-Mandly, A and Berciano, J and Gurevich, T and Giladi, N and Galitzky, M and Ory, F and Rascol, O and Kamm, C and Buerk, K and Maass, S and Gasser, T and Poewe, W and Wenning, GK},
  issn         = {0300-9564},
  keyword      = {Natural History Study,Unified MSA Rating Scale,DNA Bank,Registry,multiple system atrophy,European Multiple System Atrophy-Study Group,therapeutic trials},
  language     = {eng},
  number       = {12},
  pages        = {1677--1686},
  publisher    = {Springer},
  series       = {Journal of Neural Transmission},
  title        = {The European Multiple System Atrophy-Study Group (EMSA-SG)},
  url          = {http://dx.doi.org/10.1007/s00702-005-0328-y},
  volume       = {112},
  year         = {2005},
}