Patient Registry of the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS)

NCT ID: NCT02069509

Last Updated: 2025-10-03

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

1200 participants

Study Classification

OBSERVATIONAL

Study Start Date

2010-05-31

Study Completion Date

2024-12-31

Brief Summary

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This is a multi-centre, multi-national, prospective, observational study of Friedreich's Ataxia (FRDA) with a control group to:

* obtain natural history data on individuals affected by FRDA
* relate clinical assessments and results from proteomic analyses
* expedite identification and recruitment of participants for clinical trials
* develop and validate sensitive and reliable outcome measures for detecting onset and change over the natural course of FRDA which may also be potential outcome measures for use in future clinical trials and clinical care
* plan for future research studies

Detailed Description

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The EFACTS patient registry integrates prospectively and systematically collected clinical research data (e.g. clinical tests, demographical characteristics) with access to biological specimens (e.g. blood, urine) obtained from individuals with genetically confirmed FRDA and unrelated control research participants.

The EFACTS patient registry started out as a 4-year study and is currently running without a set end date. Eligible subjects are assessed at annual study visits on the clinical symptoms and signs of the disease. At each study visit, general clinical, motor function, cognitive, and Quality of Life assessments are administered. In addition, participants are providing bio samples for the purposes of genetic testing and for research to identify valuable biomarkers of FRDA. Biological specimens and clinical data are made available to qualified scientists within the EFACTS network whose projects are reviewed and approved by the EFACTS Steering Committee. Research projects should aim to advance scientific knowledge towards establishing clinically effective treatments that delay onset and/or slow the progression of the disease.

The EFACTS study will now be converted to a globally harmonized version of the protocol. Data from Europe will continue to be stored in a European database maintained by FARA Europe. While this is a continuation of the study for current patients, the project will be given the new study title UNIFAI to reflect the inclusion of new global sites.

Conditions

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Friedreich's Ataxia

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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Control research participants

Controls may be family members or recruited elsewhere, heterozygous GAA expansion carriers are allowed

No interventions assigned to this group

FRDA patients

with genetically confirmed diagnosis of FRDA

No interventions assigned to this group

Eligibility Criteria

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Inclusion Criteria

* Genetic diagnosis of FRDA
Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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European Friedreich's Ataxia Consortium for Translational Studies

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Jörg B. Schulz, MD

Role: PRINCIPAL_INVESTIGATOR

University Hospital Aachen, Dept. of Neurology

Paola Giunti, MD

Role: PRINCIPAL_INVESTIGATOR

University College of London, Institute of Neurology

Caterina Mariotti, MD

Role: PRINCIPAL_INVESTIGATOR

Fondazione IRCCS Istituto Neurologico Carlo Besta

Francisco J. Rodriguez de Rivera Garrido, MD

Role: PRINCIPAL_INVESTIGATOR

La Paz University Hospital, Dept. of Neurology

Alexandra Durr, MD

Role: PRINCIPAL_INVESTIGATOR

Hôpital Pitié Salpêtrière, ICM

Thomas Klopstock, MD

Role: PRINCIPAL_INVESTIGATOR

University of Munich, Dept. of Neurology with Friedrich-Baur-Institute

Sylvia Boesch, MD

Role: PRINCIPAL_INVESTIGATOR

Medical University Innsbruck, Department of Neurology

Gilles Naeije, MD

Role: PRINCIPAL_INVESTIGATOR

Université Libre de Bruxelles and Cliniques Universitaires de Bruxelles-Hôpital Erasme

Ludger Schöls, MD

Role: PRINCIPAL_INVESTIGATOR

University of Tübingen, Dept. of Neurology with Hertie-Institute for Clinical Brain Research

Thomas Klockgether, MD

Role: PRINCIPAL_INVESTIGATOR

University Hospital Bonn, Dept. of Neurology

Georgios Koutsis, MD

Role: PRINCIPAL_INVESTIGATOR

National and Kapodistrian University of Athens, Neurogenetics Unit

Sinead Murphy, MD

Role: PRINCIPAL_INVESTIGATOR

Tallaght University Hospital, Department of Neurology

Richard Walsh, MD

Role: PRINCIPAL_INVESTIGATOR

Tallaght University Hospital, Department of Neurology

Enrico Bertini, MD

Role: PRINCIPAL_INVESTIGATOR

Bambino Gesù Children's Hospital, Department of Neurosciences

Francesc Palau Martinez, MD

Role: PRINCIPAL_INVESTIGATOR

Hospital Sant Joan de Déu, Servicio de Neurología

Martin Vyhnálek, MD

Role: PRINCIPAL_INVESTIGATOR

Motol University Hospital, Centre for Hereditary Ataxias

Mathieu Anheim, MD

Role: PRINCIPAL_INVESTIGATOR

Hôpital de Hautepierre, Service de Neurologie

Bart van de Warrenburg, MD

Role: PRINCIPAL_INVESTIGATOR

STICHTING RADBOUD UNIVERSITAIR MEDISCH CENTRUM

Kathrin Reetz, MD

Role: PRINCIPAL_INVESTIGATOR

University Hospital Aachen, Dept. of Neurology

Locations

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Medical University Innsbruck, Department of Neurology

Innsbruck, , Austria

Site Status

Université Libre de Bruxelles, Hôpital Erasme, Dpt of Neurology

Brussels, , Belgium

Site Status

Motol University Hospital, Centre for Hereditary Ataxias

Prague, , Czechia

Site Status

Hôpital Pitié Salpêtrière, Bâtiment ICM

Paris, , France

Site Status

Hôpital de Hautepierre, Service de Neurologie

Strasbourg, , France

Site Status

University Hospital Aachen, Dept. of Neurology

Aachen, , Germany

Site Status

University Hospital Bonn, Dept. of Neurology

Bonn, , Germany

Site Status

University of Munich, Dept. of Neurology, Friedrich-Baur-Institut

Munich, , Germany

Site Status

University of Tübingen, Dept. of Neurodegenerative Diseases, Hertie-Institute for Clinical Brain Research

Tübingen, , Germany

Site Status

National and Kapodistrian University of Athens, Neurogenetics Unit

Athens, , Greece

Site Status

Tallaght University Hospital, Department of Neurology

Dublin, , Ireland

Site Status

Fondazione IRCCS Istituto Neurologico Carlo Besta

Milan, , Italy

Site Status

Bambino Gesù Children's Hospital, Department of Neurosciences

Roma, , Italy

Site Status

Hospital Sant Joan de Déu, Servicio de Neurología

Barcelona, , Spain

Site Status

Hospital Universitario La Paz, Servicio de Neurologia

Madrid, , Spain

Site Status

University College of London, Ataxia Centre, National Hospital for Neurology and Neurosurgery

London, , United Kingdom

Site Status

Countries

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Austria Belgium Czechia France Germany Greece Ireland Italy Spain United Kingdom

References

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Reetz K, Dogan I, Costa AS, Dafotakis M, Fedosov K, Giunti P, Parkinson MH, Sweeney MG, Mariotti C, Panzeri M, Nanetti L, Arpa J, Sanz-Gallego I, Durr A, Charles P, Boesch S, Nachbauer W, Klopstock T, Karin I, Depondt C, vom Hagen JM, Schols L, Giordano IA, Klockgether T, Burk K, Pandolfo M, Schulz JB. Biological and clinical characteristics of the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS) cohort: a cross-sectional analysis of baseline data. Lancet Neurol. 2015 Feb;14(2):174-82. doi: 10.1016/S1474-4422(14)70321-7. Epub 2015 Jan 5.

Reference Type BACKGROUND
PMID: 25566998 (View on PubMed)

Buchholz M, Pfaff M, Iskandar A, Reetz K, Schulz JB, Grobe-Einsler M, Klockgether T, Michalowsky B; EFACTS Study Group. Health-Related Quality of Life in Patients with Friedreich Ataxia Using Mobility Assistive Technologies: Limited Fit of the EQ-5D-3L Mobility Dimension. Neurol Ther. 2025 Feb;14(1):379-398. doi: 10.1007/s40120-024-00694-7. Epub 2024 Dec 30.

Reference Type DERIVED
PMID: 39738982 (View on PubMed)

Indelicato E, Reetz K, Maier S, Nachbauer W, Amprosi M, Giunti P, Mariotti C, Durr A, de Rivera Garrido FJR, Klopstock T, Schols L, Klockgether T, Burk K, Pandolfo M, Didszun C, Grobe-Einsler M, Nanetti L, Nenning L, Kiechl S, Dichtl W, Ulmer H, Schulz JB, Boesch S; European Friedreich's Ataxia Consortium for Translational Studies (EFACTS). Predictors of Survival in Friedreich's Ataxia: A Prospective Cohort Study. Mov Disord. 2024 Mar;39(3):510-518. doi: 10.1002/mds.29687. Epub 2023 Dec 23.

Reference Type DERIVED
PMID: 38140802 (View on PubMed)

Reetz K, Dogan I, Hilgers RD, Giunti P, Parkinson MH, Mariotti C, Nanetti L, Durr A, Ewenczyk C, Boesch S, Nachbauer W, Klopstock T, Stendel C, Rodriguez de Rivera Garrido FJ, Rummey C, Schols L, Hayer SN, Klockgether T, Giordano I, Didszun C, Rai M, Pandolfo M, Schulz JB; EFACTS study group. Progression characteristics of the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS): a 4-year cohort study. Lancet Neurol. 2021 May;20(5):362-372. doi: 10.1016/S1474-4422(21)00027-2. Epub 2021 Mar 23.

Reference Type DERIVED
PMID: 33770527 (View on PubMed)

Indelicato E, Nachbauer W, Eigentler A, Amprosi M, Matteucci Gothe R, Giunti P, Mariotti C, Arpa J, Durr A, Klopstock T, Schols L, Giordano I, Burk K, Pandolfo M, Didszdun C, Schulz JB, Boesch S; EFACTS (European Friedreich's Ataxia Consortium for Translational Studies). Onset features and time to diagnosis in Friedreich's Ataxia. Orphanet J Rare Dis. 2020 Aug 3;15(1):198. doi: 10.1186/s13023-020-01475-9.

Reference Type DERIVED
PMID: 32746884 (View on PubMed)

Bonnechere B, Jansen B, Haack I, Omelina L, Feipel V, Van Sint Jan S, Pandolfo M. Automated functional upper limb evaluation of patients with Friedreich ataxia using serious games rehabilitation exercises. J Neuroeng Rehabil. 2018 Oct 4;15(1):87. doi: 10.1186/s12984-018-0430-7.

Reference Type DERIVED
PMID: 30286776 (View on PubMed)

Reetz K, Dogan I, Hohenfeld C, Didszun C, Giunti P, Mariotti C, Durr A, Boesch S, Klopstock T, Rodriguez de Rivera Garrido FJ, Schols L, Giordano I, Burk K, Pandolfo M, Schulz JB; EFACTS Study Group. Nonataxia symptoms in Friedreich Ataxia: Report from the Registry of the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS). Neurology. 2018 Sep 4;91(10):e917-e930. doi: 10.1212/WNL.0000000000006121. Epub 2018 Aug 10.

Reference Type DERIVED
PMID: 30097477 (View on PubMed)

Tanguy Melac A, Mariotti C, Filipovic Pierucci A, Giunti P, Arpa J, Boesch S, Klopstock T, Muller Vom Hagen J, Klockgether T, Burk K, Schulz JB, Reetz K, Pandolfo M, Durr A, Tezenas du Montcel S; EFACTS group. Friedreich and dominant ataxias: quantitative differences in cerebellar dysfunction measurements. J Neurol Neurosurg Psychiatry. 2018 Jun;89(6):559-565. doi: 10.1136/jnnp-2017-316964. Epub 2017 Dec 26.

Reference Type DERIVED
PMID: 29279305 (View on PubMed)

Lad M, Parkinson MH, Rai M, Pandolfo M, Bogdanova-Mihaylova P, Walsh RA, Murphy S, Emmanuel A, Panicker J, Giunti P. Urinary, bowel and sexual symptoms in a cohort of patients with Friedreich's ataxia. Orphanet J Rare Dis. 2017 Sep 26;12(1):158. doi: 10.1186/s13023-017-0709-y.

Reference Type DERIVED
PMID: 28950889 (View on PubMed)

Reetz K, Dogan I, Hilgers RD, Giunti P, Mariotti C, Durr A, Boesch S, Klopstock T, de Rivera FJR, Schols L, Klockgether T, Burk K, Rai M, Pandolfo M, Schulz JB; EFACTS Study Group. Progression characteristics of the European Friedreich's Ataxia Consortium for Translational Studies (EFACTS): a 2 year cohort study. Lancet Neurol. 2016 Dec;15(13):1346-1354. doi: 10.1016/S1474-4422(16)30287-3.

Reference Type DERIVED
PMID: 27839651 (View on PubMed)

Related Links

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http://www.e-facts.eu

EFACTS Registry

Other Identifiers

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HEALTH-F2-2010- 242193

Identifier Type: -

Identifier Source: org_study_id

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