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The Huntington Study Group currently holds the registered trademark for the Unified Huntington's Disease Rating Scale. The UHDRS® is a research tool which has been developed by the HSG to provide a uniform assessment of the clinical features and course of HD. The UHDRS® has undergone extensive reliability and validity testing and has been used as a major outcome measure by the HSG in controlled clinical trials.
The purpose of this research study is to determine if a standard HD assessment (the UHDRS®) is as reliable when conducted virtually as it is when conducted at an in-person visit (vUHDRS)
The UHDRS® is comprised of five components:
Motor Assessment Cognitive Assessment Behavioral Assessment Independence Scale Functional Assessment Total Functional Capacity (TFC)
This observational study seeks to characterize and compare in-person versus TeleVisits. Fifty percent of participants will be provided computer equipment for their remote assessment and the other 50% will use their existing home-based equipment for this purpose.
TeleVisit is a method for participants to connect to the research study team remotely, via video and audio connection either on their computer or iPad.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Personal Equipment | Participants assigned to use technology they currently own | ||
| HSG Equipment | Participants provided technology by the sponsor |
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| Measure | Description | Time Frame |
|---|---|---|
| Unified Huntington's Disease Rating Scale | Total Motor Score: Minimum Score = 0 Maximum Score = 124 Higher scores indicate more impaired motor functioning | from the TeleVisit to the second In-Person Visit, up to 6 weeks |
| Unified Huntington's Disease Rating Scale | Total Functional Capacity: Minimum Score = 0 Maximum Score = 13 Greater scores indicate higher functioning | from the TeleVisit to the second In-Person Visit, up to 6 weeks |
| United Huntington's Disease Rating Scale | Independence Scale Minimum Score = 0 Maximum Score = 100 Greater scores indicate greater independence | from the TeleVisit to the second In-Person Visit, up to 6 weeks |
| United Huntington's Disease Rating Scale | Functional Assessment Minimum Score = 0 Maximum Score = 25 Higher scores indicate greater functionality | from TeleVisit to the second In-Person Visit, up to 6 weeks |
| Measure | Description | Time Frame |
|---|---|---|
| Safety of conducting the UHDRS remotely | Reported adverse events | from initial informed consent and continue until 30 days following the Participant's completion of the study |
| Unified Huntington's Disease Rating Scale |
| Measure | Description | Time Frame |
|---|---|---|
| Participant Satisfaction | Survey | from first In-Person visit through Follow-Up, up to 6 weeks |
Inclusion Criteria:
Exclusion Criteria:
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Participants will be enrolled from clinics with physicians who are members of the Huntington Study Group
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| Name | Affiliation | Role |
|---|---|---|
| Samuel Frank, MD | Huntington Study Group | Principal Investigator |
| Jody Goldstein | Huntington Study Group | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University of Alabama at Birmingham, Department of Neurology | Birmingham | Alabama | 35233 | United States | ||
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Cognitive Function:
Verbal Fluency minimum = 0, maximum is based upon participant responses Symbol Digit Modality (SDMT) minimum = 0, maximum = 110 Stroop minimum = 0, maximum is based upon participant responses Higher scores indicate greater cognitive function
| from the TeleVisit to the second In-Person Visit, up to 6 weeks |
| Unified Huntington's Disease Rating Scale | Total Motor Score Minimum Score = 0 Maximum Score = 124 Higher scores indicate more impaired motor functioning | Week 2, and Week 0 or Week 4 |
| virtual Unified Huntington's Disease Rating Scale | Feasibility of completing the vUHDRS™ using participant personal equipment vs. Huntington Study Group provided equipment. | from first In-Person visit through Follow-Up, up to 6 weeks |
| Unified Huntington's Disease Rating Scale | Behavior minimum = 0, maximum = 88 Higher scores indicate more behavioral symptoms | from the TeleVisit to the second In-Person Visit, up to 6 weeks |
| University of Arkansas for Medical Sciences |
| Little Rock |
| Arkansas |
| 72205 |
| United States |
| University of California, San Diego | La Jolla | California | 93037 | United States |
| Georgetown University | Washington D.C. | District of Columbia | 20007 | United States |
| University of South Florida, Huntington Disease Center of Excellence | Tampa | Florida | 33612 | United States |
| Northwestern University Medical Center, Department of Neurology | Chicago | Illinois | 60611 | United States |
| Rush University Medical Center | Chicago | Illinois | 60612 | United States |
| Indiana University School of Medicine | Indianapolis | Indiana | 46202 | United States |
| Hereditary Neurological Disease Center | Wichita | Kansas | 67226 | United States |
| Beth Israel Deaconess Medical Center | Boston | Massachusetts | 02215 | United States |
| Massachusetts General Hospital | Charlestown | Massachusetts | 02129 | United States |
| Washington University | St Louis | Missouri | 63110 | United States |
| Sanford Brain and Spine Clinic | Fargo | North Dakota | 58103 | United States |
| Ohio State University Medical Center | Columbus | Ohio | 43210 | United States |
| University of Pittsburgh | Pittsburgh | Pennsylvania | 15213 | United States |
| University of Texas, Health Science Center | Houston | Texas | 77030 | United States |
| University of Vermont Medical Center | Burlington | Vermont | 05401 | United States |
| ID | Term |
|---|---|
| D006816 | Huntington Disease |
| ID | Term |
|---|---|
| D001480 | Basal Ganglia Diseases |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D003704 | Dementia |
| D002819 | Chorea |
| D020820 | Dyskinesias |
| D009069 | Movement Disorders |
| D020271 | Heredodegenerative Disorders, Nervous System |
| D019636 | Neurodegenerative Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D003072 | Cognition Disorders |
| D019965 | Neurocognitive Disorders |
| D001523 | Mental Disorders |
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