Instrumented Data Exchange for Ataxia Study

Study Overview
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Brief Summary
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This research study is testing body-worn sensors to measure movement during simple tests of coordination, in order to evaluate the progression and severity of ataxia.

Official Title
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APDM Instrumented Data Exchange for Ataxia (IDEA) Study

Conditions
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Spinocerebellar Ataxia Type 1, Spinocerebellar Ataxia Type 2, Spinocerebellar Ataxia Type 3, Spinocerebellar Ataxia Type 6, Friedreich Ataxia

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: SCA 1, 2, 3, 6, and FA with mutations in the pathogenic range confirmed from genetic testing SCA: aged 18-75 years FA: aged 12-30, diagnosed between ages 5-25 community dwelling physically/cognitively capable of consenting/assenting and complying with the protocol based on investigator's judgement able to walk independently 10 yards without an assistive device able to sit or stand unassisted for 30 seconds no other neurological or musculoskeletal disorder that could affect mobility no other history of head injury, vestibular function, stroke, or other disorders that could affect mobility willing and able to participate in a 2-year study consent to be video recorded while performing study assessments Exclusion Criteria: dementia that limits subjects' ability to follow directions pain that limits mobility SCA: enrolled in a clinical drug trial

Ages Eligible for Study
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Minimum Age: 12 Years
Maximum Age: 75 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Spinocerebellar Ataxia-1<br>individuals with a genetically confirmed diagnosis of SCA-1 | |
| Spinocerebellar Ataxia-2<br>individuals with a genetically confirmed diagnosis of SCA-2 | |
| Spinocerebellar Ataxia-3<br>individuals with a genetically confirmed diagnosis of SCA-3 | |
| Spinocerebellar Ataxia-6<br>individuals with a genetically confirmed diagnosis of SCA-6 | |
| Freidreich's Ataxia<br>individuals with a genetically confirmed diagnosis of FA | |
| FA Controls<br>Healthy, age-matched controls | |
| SCA Controls<br>Healthy, age-matched controls | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| iSARA | measurements collected by wearable inertial sensors during instrumented Scale for the Assessment and Rating of Ataxia (iSARA) | 2 years | 
| SARA | Scale for the Assessment and Rating of Ataxia (SARA) measurements evaluated by clinician to measure severity of spinocerebellar ataxia through coordination, speech, stance, and gait | 2 years | 
| mFARS | modified Friedreich's Ataxia Rating Scale (mFARS) measurements evaluated by clinician compared to measure severity of Friedreich's ataxia through coordination, speech, stance, and gait | 2 years | 
| Daily Life (UChicago site ONLY) | APDM SmartSox technology used to measure daily life activity(movement, falls, etc.) of all University of Chicago subjects | 2 weeks | 
| Ataxia App on Watch/iPhone | Bi-weekly assessments completed on Ataxia Application for Apple Watches and iPhones. Data collected includes active monitoring of speech, stance, coordination, gait and patient-reported symptoms. | 2 years | 
| Patient-Reported Questionnaires of Health | Activities, Balance, and Confidence (ABC) Questionnaire; EQ-5D-5L Health Questionnaire; Modified Fatigue Impact Scale (MFIS-5); Activities of Daily Living (ADLs) | 2 years | 
| Falls Questionnaire | Automatic email sent out to subjects inquiring about any falls and near falls they have experienced during the past month | 2 years | 
| Functional Staging | Clinician evaluates severity of ataxia symptoms. | 2 years | 
| Timed 25 Foot Walk | Subjects walk for 25 feet, as quickly and safely as possible, while being timed | 2 years |