The key goals of EUROSCA-NHS is to determine and compare the rate of disease progression in SCA1, SCA2, SCA3 and SCA6 including determination of the order and occurrence of non-ataxia symptoms, assessment of activities of daily living (ADL) and quality of life (QoL), and identification of predictors of disease progression and survival.
The key goal of EUROSCA-NHS is to determine and compare the rate of disease progression in SCA1, SCA2, SCA3 and SCA6. To this end, a newly developed and validated ataxia scale (Scale for the Assessment and Rating of Ataxia, SARA) will be used. EUROSCA-NHS has a number of secondary aims including determination of the order and occurrence of non-ataxia symptoms, assessment of activities of daily living (ADL) and quality of life (QoL), and identification of predictors of disease progression and survival. Substudies will deal with the development of brain atrophy, as assessed by magnetic resonance imaging (MRI), progression of peripheral neuropathy, as assessed by nerve conduction studies, and specific clinical aspects of SCA.
Study Type
OBSERVATIONAL
Enrollment
400
Department of Neurology, Medical University, Innsbruck
Innsbruck, Austria
Scale for the assessment and rating of ataxia (SARA)
Progression of ataxia is measured using a newly developed and validated ataxia scale, SARA. SARA was evaluated in two large validation trials performed by the EUROSCA clinical group and was found to be easy to use, reliable, and valid.
Time frame: Patients are first seen at a baseline visit, followed by annual visits for 3 years scheduled ± 3 months around the specified time point. After the initial 3 year observation period, visits are done at irregular intervals each time they went to hospital.
Disease stages
Disease stages are measured using the 5 point scale ranging from 0 to 4 proposed by Klockgether et al., 1998.
Time frame: Patients are first seen at a baseline visit, followed by annual visits for 3 years scheduled ± 3 months around the specified time point. After the initial 3 year observation period, visits are done at irregular intervals each time they went to hospital.
Inventory of non-ataxia signs (INAS)
The occurrence of accompanying non-ataxia symptoms is recorded using INAS. In the SARA validation trials, INAS was applied to a large number of SCA patients. Statistical evaluation showed good reliability.
Time frame: Patients are first seen at a baseline visit, followed by annual visits for 3 years scheduled ± 3 months around the specified time point. After the initial 3 year observation period, visits are done at irregular intervals each time they went to hospital.
UHDRS part IV
Functional disability in ADL is assessed using the Functional assessment part of the Unified Huntington's Disease Rating Scale (UHDRS) (Huntington Study Group, 1996). This 25-item assessment has been used in SCA patients throughout the SARA validation study with good practicality.
Time frame: Patients are first seen at a baseline visit, followed by annual visits for 3 years scheduled ± 3 months around the specified time point. After the initial 3 year observation period, visits are done at irregular intervals each time they went to hospital.
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Université Libre de Bruxelles (ULB), Neurology Service - ULB Hôpital Erasme, ULB Laboratory of Experimental Neurology
Brussels, Belgium
ACTIVE_NOT_RECRUITINGHôpital de la Pitié-Salpêtrière, Département de Génétique
Paris, France
ACTIVE_NOT_RECRUITINGDepartment of Neurology, St. Josef Hospital, University Hospital of Bochum
Bochum, Germany
ACTIVE_NOT_RECRUITINGDepartment of Neurology, University of Bonn
Bonn, Germany
RECRUITINGDepartment of Neurology, University Clinic Essen, University of Duisburg-Essen
Essen, Germany
ACTIVE_NOT_RECRUITINGDepartment of Neurology, University of Frankfurt
Frankfurt, Germany
ACTIVE_NOT_RECRUITINGDepartment of Neurodegeneration and Hertie-Institute for Clinical Brain Research, University of Tübingen
Tübingen, Germany
ACTIVE_NOT_RECRUITINGDepartment of Medical Genetics, University of Pecs
Pécs, Hungary
ACTIVE_NOT_RECRUITINGDepartment of Neurology, Zala County Hospital
Zalaegerszeg, Hungary
ACTIVE_NOT_RECRUITING...and 6 more locations
EQ-5D
Health related Quality of life is assessed using EQ-5D, a generic instrument that has been developed and validated by the EuroQuol Group (1990) and is available in validated translations for use as a questionnaire.
Time frame: Patients are first seen at a baseline visit, followed by annual visits for 3 years scheduled ± 3 months around the specified time point. After the initial 3 year observation period, visits are done at irregular intervals each time they went to hospital.
PHQ-9
Assessment of depressive symptoms is done using a validated 9-item short form of the Patient Health Questionnaire (PHQ), a questionnaire that has been developed to screen for psychiatric co-morbidity in unselected populations (Spitzer et al. 1999).
Time frame: Patients are first seen at a baseline visit, followed by annual visits for 3 years scheduled ± 3 months around the specified time point. After the initial 3 year observation period, visits are done at irregular intervals each time they went to hospital.