Try our beta test site
IMPORTANT: Listing of a study on this site does not reflect endorsement by the National Institutes of Health. Talk with a trusted healthcare professional before volunteering for a study. Read more...

Measuring Neurological Impairment and Functional Visual Assessment In Spinocerebellar Ataxias

This study has been completed.
University of Texas
Information provided by:
University of Mississippi Medical Center Identifier:
First received: April 1, 2008
Last updated: April 28, 2009
Last verified: April 2009

Measuring the various difficulties patients with spinocerebellar ataxias (SCA) report in an accurate manner is important to be able to test any therapy that may be developed. As basic research identifies some therapy of this type, clinicians are planning studies that can either prove or disprove that such treatments actually have an effect. Walking problems and problems with eye movements that can give rise to visual complaints are common in the SCA's.

Existing neurological scales such as the "SARA" are based on the usual neurological examination items that can carry a degree of subjective bias. Also the intervals between numbers on such scores often do not carry the same "weight" so that the difference between a score of 1 and 2 may not be equal to difference between 2 and 3. Lastly, such scales done in the clinic setting capture only a brief period of a patient's day. We propose that examination of home based gait monitoring, timed tests of motor function and quantitative measures of visual problems in patients with SCA are more useful in measuring the disability in these patients.

Spinocerebellar Ataxia

Study Type: Observational
Study Design: Observational Model: Cohort
Time Perspective: Prospective
Official Title: Utility Of Home Based Gait Monitoring, Performance Scores And Functional Visual Assessment In Spinocerebellar Ataxias (SCA)

Resource links provided by NLM:

Further study details as provided by University of Mississippi Medical Center:

Primary Outcome Measures:
  • Home based gait data vs SARA index [ Time Frame: 0, 6, 12, 24 months ]

Secondary Outcome Measures:
  • Barthel Index [ Time Frame: 0, 6, 12, 24 months ]
  • Visual function- acuity, contrast, stereo, color [ Time Frame: 0, 6, 12, 24 months ]
  • Ocular Motility defects- phoria/tropia, vergence amplitudes [ Time Frame: 0, 6, 12, 24 months ]

Enrollment: 20
Study Start Date: February 2008
Study Completion Date: February 2009
Primary Completion Date: February 2009 (Final data collection date for primary outcome measure)
Detailed Description:

Spinocerebellar ataxias (SCA's) are relatively rare, inherited disorders of the cerebellum that cause inexorably progressive imbalance and incoordination as well as speech problems. Recent molecular genetic studies have raised hopes for meaningful treatments for these conditions. Before one can embark on therapeutic studies on the SCA's it is essential that we have validated instruments for measuring disease progression. Rating scales based on neurological examinations have been reported recently but their sensitivity to change and reproducibility are still being examined. In an attempt to produce readily quantifiable and sensitive measures of motor function in the SCA's, we are proposing to examine the utility of home based gait monitoring, timed tests of motor function and quantitative measures of visual problems in patients with SCA's. These measurements which will produce continuous rather than categorical variables, and may be subject to less arbitrariness on the part of the examiner, are hypothesized to be not only valid measures but also more sensitive.

The overall aim of the project is to assess the utility of home based gait monitoring, timed performance measures and functional visual testing as measures to monitor disease progression in SCA's. The concurrent validity of these measures will be tested by correlating them with an established examination based ataxia scale (Scale for assessment and rating of ataxia: SARA), an activity of daily living scale (Barthel index) and functional staging.

Aim 1: Hypothesis: Motor impairment in SCA's can be assessed validly in the home setting by utilizing the step activity monitor (SAM).

Aim 2: Hypothesis: Motor impairment in SCA's can be further quantified using performance measures: the 9-hole peg board test and the timed 25 foot walk.

Aim 3: Hypothesis: Visual problems reported by patients with SCA's can be quantified by low contrast vision testing, stereoacuity measures, color vision and vergence amplitudes utilizing well-established ophthalmologic techniques.

Aim 4: Hypothesis: The neurological impairment as measured by these measures will correlate with each other, with scores on SARA, with the Barthel index score and with functional staging.

Aim 5: Hypothesis: Ataxia grading using the SAM, timed performance measures and functional visual scores will be more sensitive than SARA for measuring disease progression


Ages Eligible for Study:   18 Years to 80 Years   (Adult, Senior)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Non-Probability Sample
Study Population
Patients attending the Ataxia Clinic

Inclusion Criteria:

  1. Patients with the diagnosis of Spinocerebellar Ataxia (Type 1,2,3,6,7,8 and 10) will be selected from those attending the ataxia clinic in the Neurology Department at University of Mississippi Medical Center
  2. Patients will be 18years or older in age
  3. All patients are able to walk with or without assisted devices

Exclusion Criteria:

  1. Patients less than 18years.
  2. Patients who are wheelchair bound
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its identifier: NCT00654251

United States, Mississippi
University of Mississippi Medical Center
Jackson, Mississippi, United States, 39211
United States, Texas
University of Texas Medical Branch
Galveston, Texas, United States, 77555
Sponsors and Collaborators
University of Mississippi Medical Center
University of Texas
Study Chair: James J Corbett, MD University of Mississippi Medical Center
Study Director: S H Subramony, MD University of Texas
Study Director: Sachin Kedar, MD University of Mississippi Medical Center
  More Information

Responsible Party: James J Corbett, University of Mississippi Medical Center Identifier: NCT00654251     History of Changes
Other Study ID Numbers: 2007-0228
Study First Received: April 1, 2008
Last Updated: April 28, 2009

Keywords provided by University of Mississippi Medical Center:
Spinocerebellar ataxia
Machado-Joseph disease
Gait monitoring
Functional vision assessment

Additional relevant MeSH terms:
Cerebellar Ataxia
Spinocerebellar Ataxias
Spinocerebellar Degenerations
Neurologic Manifestations
Nervous System Diseases
Signs and Symptoms
Cerebellar Diseases
Brain Diseases
Central Nervous System Diseases
Spinal Cord Diseases
Heredodegenerative Disorders, Nervous System
Neurodegenerative Diseases
Genetic Diseases, Inborn processed this record on May 25, 2017