Dysmetria in Motor Function in SCA: Mechanisms and Rehabilitation
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 19
- 试验地点
- 2
- 主要终点
- Change in the Location of the Movement Endpoint Relative to the Target in the Motor Task
研究概览
简要总结
The purpose of this research study is to investigate how the brain and motor behavior changes both in individuals with spinocerebellar ataxia and healthy individuals, and to assess whether a therapeutic intervention reduces levels of uncoordinated movement and improves motor function in spinocerebellar ataxia (SCA).
详细描述
Thirty individuals who have been diagnosed with either Spinocerebellar Ataxia - 1 (SCA1), Spinocerebellar Ataxia - 3 (SCA3), or Spinocerebellar Ataxia - 6 (SCA6) will be recruited for this study. Participants will be randomly assigned to a best medical management (BMM / control) group and an error-reduction group. All participants will visit the lab twice for testing one month apart. Participants in the control group will not train between the pre- and post-test time.
The error-reduction intervention will be a 4-week home-based program. Investigators will use a novel, custom designed computer interface. Participants will perform goal-directed movements with each leg to targets in a 3D virtual environment designed to emphasize accurate movements. The goal-directed leg movements (similar to leg press) will be performed seated and require hip, knee, and ankle joint control. Leg movement will be detected using the LeapMotion sensor (Leap Motion Inc. San Francisco, CA), a device that supports hand, and finger / tool motions as input, similar to a mouse, but requiring no contact. Spatial endpoint errors will be quantified in 3D space by comparing the endpoint location of the foot trajectory (extending from the big toe) and the virtual location of the target. Time endpoint errors will be quantified by comparing the timing of the foot trajectory and the required time to target.
The length of the intervention will be 4 weeks. Each participant will train 4 days a week for ~1 hour per day. Within a week the task difficulty will increase by changing the presentation of the target from a predictable to an unpredictable location, by increasing movement speed requirements and by changing target size. Targets will be made predictable by identifying them prior to the cue for movement onset (target turning green). Specifically, there will be a flashing dotted line around the target prior to the target turning green. Targets will be made unpredictable by not providing any indication of the target location prior to the target turning green. Movement speed will be quantified from the voluntary movement onset of the leg (no reaction time) to the movement end.The movement speed requirements will be increased within a week and participants will learn to execute faster movements from the feedback after each trial. The size of the target will be progressively reduced during the 4 weeks.
All individuals in the study will receive a pre- and post-test assessment using the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA). The individual sections of the ICARS (e.g. Kinetic section) and SARA will be quantified.
In addition, leg dysmetria will be quantified using a custom-made goal-directed movement protocol. Specifically, participants will perform unloaded ankle dorsiflexion movements and attempt to reach a space-time target. The primary outcomes will be position and time errors.
研究设计
- 研究类型
- Interventional
- 分配方式
- Randomized
- 干预模型
- Parallel
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 年龄范围
- 21 Years 至 85 Years（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •DNA diagnosis of SCA1, SCA3, or SCA6
- •phenotype consistent with the DNA diagnosis
- •ability to walk 7 meters
- •the age of 21-85 years
- •capable of providing informed consent and complying with the trial procedures
排除标准
- •Known recessive, X-linked or mitochondrial ataxias or any other type of ataxia
- •Concomitant disorder(s) that affect ataxia measures used in this study
- •Cognitive status on the Montreal Cognitive Assessment < 24
- •Patients who have any type of implanted electrical device (such as a cardiac pacemaker or a neurostimulator), or a certain type of metallic clip in their body (i.e., an aneurysm clip in the brain), and are not eligible for participation in the MRI portion of the study
- •Individuals who are claustrophobic
- •Women who are or might be pregnant and nursing mothers
- •Individuals with psychiatric disorders or dementia, along with other neurological and orthopedic problems that impair hand movements and walking
研究组 & 干预措施
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: Error-reduction (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: International Cooperative Ataxia Rating Scale (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: Scale for the Assessment and Rating of Ataxia (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: Beck Depression Inventory, 2nd Ed (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: Stroop (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: Purdue Pegboard (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: Brief Test of Attention (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: 6-minute Walk (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: Hand Grip Dynamometer (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: Montreal Cognitive Assessment (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: Physical Performance Function (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: Biomechanical Assessments of Dysmetria (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: Neurophysiological assessment of brain activity (Behavioral)
Error-reduction
The participants in the error-reduction group will participate in a 4-week home-based training intervention during the month between their pre- and post-test visits. During pre- and post- training visits, Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA), Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait analysis tests will be administered. Also biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted to evaluate the impact of the training on SCA individuals.
干预措施: Biomechanical gait analysis (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: International Cooperative Ataxia Rating Scale (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: Scale for the Assessment and Rating of Ataxia (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: Beck Depression Inventory, 2nd Ed (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: Stroop (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: Purdue Pegboard (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: Brief Test of Attention (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: 6-minute Walk (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: Hand Grip Dynamometer (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: Montreal Cognitive Assessment (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: Physical Performance Function (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: Biomechanical Assessments of Dysmetria (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: Neurophysiological assessment of brain activity (Behavioral)
Best Medical Management
The participants in the best medical management group will undergo identical testing sessions (two visits one month apart) as those in the error-reducing group but will not receive the 4-week error reducing intervention. They will be administered the International Cooperative Ataxia Rating Scale (ICARS) and the Scale for the Assessment and Rating of Ataxia (SARA) assessments and the following tests: Purdue Pegboard, Brief Test of Attention, 6-minute Walk, Hand Grip Dynamometer, Physical Performance Function, Digit Span, SARA, Montreal Cognitive Assessment, Beck Depression Inventory 2nd Ed, Stroop and biomechanical gait. Biomechanical assessments of dysmetria and neurophysiological assessment of brain activity will be conducted at both visits.
干预措施: Biomechanical gait analysis (Behavioral)
结局指标
主要结局
Change in the Location of the Movement Endpoint Relative to the Target in the Motor Task
时间窗: Change from Baseline to 1 month
Assessment of the subject's ability to stay on target during the motor task. The task is a goal-directed movement that aims to match a spatial target in a specific time(time target).
次要结局
- Change in Blood-oxygen-level-dependent(BOLD) Activity of Motor Cortex(Change from Baseline to 1 month)
- International Cooperative Ataxia Rating Scale(ICARS) Assessment(Change from Baseline to 1 month)
- Change in Motor Unit Discharge Rate Variability(Change from Baseline to 1 month)