Huntington's Disease
Conditions
Keywords
Huntington's Disease, Chorea, Gait, Balance
Brief summary
In individuals with Huntington's disease (HD), chorea may contribute to balance problems and difficulties with walking, sit to stand transfers and stair climbing that in turn may contribute to high fall rates. Xenazine (tetrabenazine) is a monoamine-depleting drug that is commonly used to reduce chorea. The purpose of this study is to compare: 1) spatial and temporal gait measures, 2) performance on functional mobility measures, and 3) amount of daily walking activity before and after administration of Xenazine in individuals with HD. It is hypothesized that the use of Xenazine to decrease chorea will improve functions of 1) gait, 2) sit-to-stand transfers 3) stair climbing and 4) overall daily physical activity and function.
Detailed description
Individuals with HD who are ambulatory and are either already taking Xenazine or have been newly prescribed the medication will be recruited for this prospective open-label study. A variety of motor assessments will be preformed while subject are taking Xenazine or after a period off of the medications. These tests include: Unified Huntington's Disease Rating Scale (UHDRS) motor section, GAITRite forward walking, Tinetti Mobility Test, Timed Sit-to-Stand Test, Timed Stair Climb Test, Rhomberg test. A subset of subjects will wear an activity monitoring device on the wrist of the non-dominant hand for 5 consecutive days and nights.
Interventions
None listed
Sponsors
Lundbeck LLC
Ohio State University
Study design
Observational model
CASE_CONTROL
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Huntington's Disease * Able to ambulate independently
Exclusion criteria
* Other orthopedic or neurological disorder that affects gait or balance * Pregnancy * Chorea score \<10 prior to initiation of medication. * Inability to provide informed consent
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Tinetti Mobility Test Score | >18 hours off Stable Dose of Tetrabenazine and at 2 hours after resumption of Tetrabenazine | The Tinetti Mobility Test is a clinical test used to assess balance and gait. The Balance sub-score ranges from 0-16 (with 16 reflecting better balance) while the Gait sub-score ranges from 0-12 (with 12 reflecting better gait parameters). The Total Tinetti Mobility Test Score (TMT) is a sum of the two sub-scores with a maximum score of 28. The higher the score the better the gait and balance performance. A comparison of scores off regular stable dose of tetrabenazine for \>18 hours with the performance two hours after resuming tetrabenazine was made. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Five Times Sit to Stand Test | >18 hours off Stable Dose of Tetrabenazine and at 2 hours after resumption of Tetrabenazine | Subjects are asked to sit in a chair with their arms across their chests and asked to stand and sit five times in a row. The time it takes to complete 5 sit to stand cycles is timed with a stop watch. Comparison is made when off stable dose of tetrabenazine for \>18 hours to performance 2 hours after resumption of tetrabenazine. Lower time scores are associated with better balance. |
Other
| Measure | Time frame | Description |
|---|---|---|
| Six Condition Romberg Test | >18 hours off Stable Dose of Tetrabenazine and at 2 hours after resumption of Tetrabenazine | The Six Condition Romberg Test is used assess static balance. Subjects are tested standing with their arms crossed over their chests and are assessed for 30 seconds in the following 6 conditions: 1) feet together, 2) feet together eyes closed, 3) feet aligned in tandem heel-to-toe position eyes open, 4) feet aligned in tandem heel-to-toe position eyes closed, 5) standing in tandem position while counting backwards by 3's from 100 with eyes open, 6) standing in tandem position while counting backwards by 3's from 100 with eyes closed. Comparison of performances were made when off stable dose of tetrabenazine for \> 18 hours to performance 2 hours after resumption of tetrabenazine. If a participant could not hold a stance for the full 30 seconds, then that component of the Romberg test ended at that point with the time of that component scored in seconds; 30 seconds being the maximum score for each of the 6 tests. The total score was calculated as a sum of all of the 6 subset scores. |
Countries
United States
Participant flow
Participants by arm
| Arm | Count |
|---|---|
| Individuals on Stable Doses of Tetrabenazine 11 Individuals on stable doses of tetrabenazine were studied while off medication and then following resumption of medication. | 11 |
| Total | 11 |
Baseline characteristics
| Characteristic | Individuals on Stable Doses of Tetrabenazine |
|---|---|
| Age, Categorical <=18 years | 0 Participants |
| Age, Categorical >=65 years | 1 Participants |
| Age, Categorical Between 18 and 65 years | 10 Participants |
| Age, Continuous | 52.2 years STANDARD_DEVIATION 10 |
| Region of Enrollment United States | 11 participants |
| Sex: Female, Male Female | 9 Participants |
| Sex: Female, Male Male | 2 Participants |
Adverse events
| Event type | EG000 affected / at risk |
|---|---|
| deaths Total, all-cause mortality | — / — |
| other Total, other adverse events | 0 / 11 |
| serious Total, serious adverse events | 0 / 11 |
Outcome results
Primary
Tinetti Mobility Test Score
The Tinetti Mobility Test is a clinical test used to assess balance and gait. The Balance sub-score ranges from 0-16 (with 16 reflecting better balance) while the Gait sub-score ranges from 0-12 (with 12 reflecting better gait parameters). The Total Tinetti Mobility Test Score (TMT) is a sum of the two sub-scores with a maximum score of 28. The higher the score the better the gait and balance performance. A comparison of scores off regular stable dose of tetrabenazine for \>18 hours with the performance two hours after resuming tetrabenazine was made.
Time frame: >18 hours off Stable Dose of Tetrabenazine and at 2 hours after resumption of Tetrabenazine
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Individuals 2 Hours After Resuming Tetrabenazine | Tinetti Mobility Test Score | 19.91 Total Tinetti Mobility Test Score (TMT) | Standard Deviation 3.53 |
| Individuals Off Their Stable Dose of Tetrabenazine for > 18 hr | Tinetti Mobility Test Score | 17.09 Total Tinetti Mobility Test Score (TMT) | Standard Deviation 4.04 |
Secondary
Five Times Sit to Stand Test
Subjects are asked to sit in a chair with their arms across their chests and asked to stand and sit five times in a row. The time it takes to complete 5 sit to stand cycles is timed with a stop watch. Comparison is made when off stable dose of tetrabenazine for \>18 hours to performance 2 hours after resumption of tetrabenazine. Lower time scores are associated with better balance.
Time frame: >18 hours off Stable Dose of Tetrabenazine and at 2 hours after resumption of Tetrabenazine
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Individuals 2 Hours After Resuming Tetrabenazine | Five Times Sit to Stand Test | 12.61 seconds to complete 5 sit to stand cycle | Standard Deviation 3 |
| Individuals Off Their Stable Dose of Tetrabenazine for > 18 hr | Five Times Sit to Stand Test | 15.52 seconds to complete 5 sit to stand cycle | Standard Deviation 3.81 |
p-value: 0.009t-test, 2 sided
Other Pre-specified
Six Condition Romberg Test
The Six Condition Romberg Test is used assess static balance. Subjects are tested standing with their arms crossed over their chests and are assessed for 30 seconds in the following 6 conditions: 1) feet together, 2) feet together eyes closed, 3) feet aligned in tandem heel-to-toe position eyes open, 4) feet aligned in tandem heel-to-toe position eyes closed, 5) standing in tandem position while counting backwards by 3's from 100 with eyes open, 6) standing in tandem position while counting backwards by 3's from 100 with eyes closed. Comparison of performances were made when off stable dose of tetrabenazine for \> 18 hours to performance 2 hours after resumption of tetrabenazine. If a participant could not hold a stance for the full 30 seconds, then that component of the Romberg test ended at that point with the time of that component scored in seconds; 30 seconds being the maximum score for each of the 6 tests. The total score was calculated as a sum of all of the 6 subset scores.
Time frame: >18 hours off Stable Dose of Tetrabenazine and at 2 hours after resumption of Tetrabenazine
| Arm | Measure | Value (MEAN) | Dispersion |
|---|---|---|---|
| Individuals 2 Hours After Resuming Tetrabenazine | Six Condition Romberg Test | 83.08 Six Condition Romberg Test score | Standard Deviation 27.57 |
| Individuals Off Their Stable Dose of Tetrabenazine for > 18 hr | Six Condition Romberg Test | 73.26 Six Condition Romberg Test score | Standard Deviation 16.29 |
p-value: 0.114t-test, 2 sided