• Development of the Virtual Unified Huntington's Disease Rating Scale

  NCT05748288

  CompletedObservationalUpdated Sep 28, 2023
• Longitudinal Endpoint Assessment of Disease Burden in HD

  NCT06475898

  RecruitingObservationalUpdated Sep 10, 2025
• Evaluation of Three Tests to Assess Social Cognition in Huntington Disease

  NCT07253038

  RecruitingObservationalUpdated Mar 11, 2026
• REGISTRY-JHD - an Observational Study of the European Huntington's Disease Network (EHDN)

  NCT01590602

  CompletedObservationalUpdated Sep 15, 2017
• Huntington's Disease Biobank: Advancing Remote Monitoring and Deep Phenotyping

  NCT06941662

  RecruitingObservationalUpdated Apr 24, 2025
• Enroll -HD: A Prospective Registry Study in a Global Huntington's Disease Cohort

  NCT01574053

  RecruitingObservationalUpdated Feb 28, 2024

ClinicalTrials.gov ID

NCT04370470

Lead Sponsor

CHDI Foundation, Inc.OTHER

Overall Status

Unknown status

Study Type

Observational

Enrollment

340Estimated

Last Update Posted

May 1, 2020Actual

Start Date

Sep 2020Estimated

Primary Completion Date

Mar 2022Estimated

Completion Date

Mar 2022Estimated

Official Title

Development of Assessments for Later Stage Huntington's Disease: UHDRS Structured Interview of Function and HD Clinical Status Questionnaire

Brief Summary

Later Stage HD Assessments (LSA) is an observational, multinational study aiming at developing two assessments that can be used to measure critical milestones and events during the later stages of Huntington's disease (HD). An important aspect of the evaluation will be to assess whether the assessments can be administered to a companion either in-person or remotely (i.e. by phone contact with the companion). Therefore, these assessments will be evaluated for their internal consistency, reliability and validity. Once established, these assessments may be incorporated into a large scale, global observational study of HD and/or other HD clinical studies as well as use them for planning clinical trials.

Detailed Description

The primary objective of this study is to evaluate the internal consistency, reliability and validity of two assessments: (1) UHDRS Structured Interview of Function (SIF) that incorporates the UHDRS Total Functional Capacity, Functional Assessment Scale, and Independence Scale (UHDRS TFC, FAS and IS), and (2) the HD Clinical Status Questionnaire (HDCSQ). There will be two study parts: Part 1 will measure the performance of the UHDRS SIF assessment compared to the original UHDRS TFC, FAS and IS using a cross-over design by collecting data from the Manifest HD gene expansion carrier participants (Manifest HDGEC Participants) and their companions (Companion Participants) via an in-person baseline visit and a follow-up visit by phone. Part 2 will assess the UHDRS SIF and the HDCSQ using a cross-sectional design by collecting data from the Companion Participants via a follow-up visit by phone. Participants of Part 1 are eligible to participate in Part 2.

• Huntington Disease

Ages Eligible for Study

Adult (18-64)Older Adult (65+)

Sexes Eligible for Study

All

Accepts Healthy Volunteers

No

Eligibility Criteria

Inclusion Criteria:

Participants must meet all the following inclusion criteria to participate in this study:

All Participants

1. Individual of either gender
2. Fluent in English
3. Must be willing and able to provide informed consent or have a legal representative willing and able to provide assent

Manifest HDGEC Participants

1. Must be an active participant in the Enroll-HD study. An active participant is defined as a participant who has successfully enrolled in the Enroll-HD study and completed the Enroll-HD study baseline visit.
2. Age≥20 years
3. CAG repeat length≥36
4. DCL=4
5. Inclusion score of 16 at the time of the participant's most recent Enroll-HD study visit (for inclusion score calculation, see Appendix A - Inclusion Score Calculation) Companion Participants

1. Age≥18 years 2. A person who, in his/her opinion, has sufficient interface and knowledge of the Manifest HDGEC Participant's capabilities and daily activities 3. Acceptable to the Manifest HDGEC Participant and the Site Investigator or the Site Investigator's designees

Exclusion Criteria:

People who meet the following criteria will be excluded from participating in this study:

1. Individuals with choreic movement disorders in the context of a laboratory verified non-expansion mutation for the Huntingtin gene
2. Manifest HDGEC Participants with Juvenile-onset HD (rater estimate of age of onset <20 years old)

Observational Model

Other

Time Perspective

Prospective

Lead Sponsor

CHDI Foundation, Inc.OTHER

annemieke.fox@poole.nhs.uk