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F24 Development of assessments for later stage Huntington’s disease: HD-structured interview of function and HD clinical status questionnaire
  1. MW Roché1,
  2. S Capodarca2,
  3. P Winkowski1,
  4. OJ Handley2,
  5. S Frank3,
  6. RLM Fuller1,
  7. M Orth4,
  8. J Levey1,
  9. E Neacy1,
  10. C Sampaio1
  1. 1CHDI Management/CHDI Foundation, Princeton, New Jersey, USA
  2. 2European Huntington’s Disease Network, Europe
  3. 3Beth Israel Deaconess Medical Center, Boston, MA, USA
  4. 4Siloah AG, Bern, Switzerland


Background There is a need for validated assessments for patients with later-stage HD.

Aims This study aims to evaluate the clinimetric properties for two such measures: the HD Structured Interview of Function (HD-SIF) and HD Clinical Status Questionnaire (HDCSQ). Both assessments are administered to a companion either in-person or remotely (i.e. by phone contact with the companion), and the properties of these tests will be evaluated in a two-part study using the methods of Classical Test Theory (CTT) and Item Response Theory (IRT).

Methods 170 dyads of people with Huntington’s disease and their Companion Participants will be enrolled. The study includes two parts. In Part 1, we will use the methods of CTT to evaluate the HD-SIF, a structured interview designed to gather information for making ratings on the UHDRS ‘99 functional scales (TFC, FAS and IS). In Part 2, we will use the methods of CTT and IRT to assess the clinimetric properties of the HDCSQ, a questionnaire designed specifically to capture information on disease milestones that occur during the later stages of HD, and the HD-SIF.

Status and outlook Four US sites, have recruited 15 dyads over a 5-month period. Preliminary results from Part 1 will be available by the end of 2022. Part 2 began in June 2022 with preliminary results expected by the end of 2023. Upon establishing the clinimetric properties of the scales, these assessments may be used for planning studies or incorporated into observational and interventional studies of HD.

  • Function
  • clinician reported outcome
  • assessment
  • clinimetrics
  • later stage Huntington’s disease

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