Friedreich Ataxia, Spinocerebellar Ataxia Type 1, Spinocerebellar Ataxia Type 2, Spinocerebellar Ataxia Type 3, Spinocerebellar Ataxia Type 6
Conditions
Brief summary
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.
Interventions
None listed
Sponsors
Pfizer
Biogen
APDM Wearable Technologies
University of Chicago
Study design
Observational model
CASE_CONTROL
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
12 Years to 75 Years
Healthy volunteers
Yes
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| SARA | 2 years | 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 |
| iSARA | 2 years | measurements collected by wearable inertial sensors during instrumented Scale for the Assessment and Rating of Ataxia (iSARA) |
| mFARS | 2 years | 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 |
| Daily Life (UChicago site ONLY) | 2 weeks | APDM SmartSox technology used to measure daily life activity(movement, falls, etc.) of all University of Chicago subjects |
| Ataxia App on Watch/iPhone | 2 years | 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. |
| Patient-Reported Questionnaires of Health | 2 years | Activities, Balance, and Confidence (ABC) Questionnaire; EQ-5D-5L Health Questionnaire; Modified Fatigue Impact Scale (MFIS-5); Activities of Daily Living (ADLs) |
| Falls Questionnaire | 2 years | Automatic email sent out to subjects inquiring about any falls and near falls they have experienced during the past month |
| Functional Staging | 2 years | Clinician evaluates severity of ataxia symptoms. |
| Timed 25 Foot Walk | 2 years | Subjects walk for 25 feet, as quickly and safely as possible, while being timed |
Countries
United States
Outcome results
None listed