Training in Ataxia - Individuals With Degenerative Cerebellar Diseases

This is to measure ataxia severity. The Scale for the Assessment and Rating of Ataxia (SARA) will be administered before and after training. SARA is an 8-item performance based scale, yielding a total score of 0 (no ataxia) to 40 (most severe ataxia) - with higher scores indicating more severe ataxia. The scores are based on patient performance of gait, stance, sitting, speech disturbance, finger chase, nose-finger test, fast alternating hand movements and heel-shin slide. The change in score from baseline to 6 months, 9 months, and 12 months will be reported.

This is to measure average time to complete 8-meter walk test. Participants will walk 8 meters as fast as possible three different times. Gait will be reported as meters per second (m/s).

The dynamic gait index (DGI) will be performed to assess balance. Patients will be asked to walk 20 feet and conditions such as speed and head position will be varied as previously described. The examiner will then grade the subject's movement on a four-point ordinal scale, ranging from 0 (lowest level of function) to 3 (highest level of function). The total score range is 0 to 24, with higher scores indicating better dynamic balance and functional mobility.

The Timed Up and Go will be performed to assess balance. Once the tester says "Go", participants will stand from seated and walk around a cone that is 3 meters away, then walk back to the chair and sit back down. Participants will be timed from the moment the tester says "Go" until seated again.

The FSS is a nine-item questionnaire that measures the severity of fatigue. Each item is rated on a 7-point Likert scale, from 1 (strongly disagree) to 7 (strongly agree). Scores for all nine items are summed to calculate the total score, which ranges from 9 to 63. Higher scores indicate greater fatigue severity.

The WHOQOL-BREF measures self-perceived quality of life across four domains: Physical Health, Psychological, Social Relationships, and Environment. Scores are derived from a 5-point Likert scale, with higher scores indicating better quality of life in that specific area. To calculate the domain scores, the mean score for items within each domain is multiplied by 4, and these scores are transformed to a 0 - 100 scale for interpretation.

The WHOQOL-BREF measures self-perceived quality of life across four domains: Physical Health, Psychological, Social Relationships, and Environment. Scores are derived from a 5-point Likert scale, with higher scores indicating better quality of life in that specific area. To calculate the domain scores, the mean score for items within each domain is multiplied by 4, and these scores are transformed to a 0 - 100 scale for interpretation.

The WHOQOL-BREF measures self-perceived quality of life across four domains: Physical Health, Psychological, Social Relationships, and Environment. Scores are derived from a 5-point Likert scale, with higher scores indicating better quality of life in that specific area. To calculate the domain scores, the mean score for items within each domain is multiplied by 4, and these scores are transformed to a 0 - 100 scale for interpretation.

The WHOQOL-BREF measures self-perceived quality of life across four domains: Physical Health, Psychological, Social Relationships, and Environment. Scores are derived from a 5-point Likert scale, with higher scores indicating better quality of life in that specific area. To calculate the domain scores, the mean score for items within each domain is multiplied by 4, and these scores are transformed to a 0 - 100 scale for interpretation.

Diffusion data will be preprocessed for motion and corrected for geometrical distortion using ExploreDTI. For each participant, the bmatrix will be reoriented to provide a more accurate estimate of diffusion tensor orientations. Diffusion tensor estimation will be performed using a non-linear least square fitting method. FA and Mean Diffusivity (MD) maps will be generated. Whole brain tractography will be performed using all brain voxels with FA ≤ 0.2 as seed region.

To determine cerebellar volume, each T1 scan will be visually inspected to ensure inclusion of only minimal movement artifacts. All images will be processed in a blinded manner in order to maintain accuracy and consistency of volume calculation. Regional cerebellar volumes will be calculated using the SUIT toolbox of the SPM12 software.

This measure will be a primary outcome for Aim 3. The anatomical and functional data will be pre-processed and analyzed using Statistical Parametric Mapping (SPM12) and the CONN toolbox Version 14p.