Huntington's Disease movement disorder
Conditions
Interventions
None listed
Sponsors
Leids Universitair Medisch Centrum
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: All eligible participants must meet the following inclusion criteria: - Have a confirmed CAG-expansion of *36 in the HTT gene. - Be employed, or have been employed 2 years prior to assessment, in any capacity (e.g. full time (paid job for 36 hours or more), part-time (paid job for less than 36 hours) or volunteer (unpaid job)). - Be at least 18 years of age. - Be at most 64 years of age, due to the longitudinal set up of the research and the age of retirement in the Netherlands.
Exclusion criteria
Exclusion criteria: All eligible participants must meet none of the following exclusion criteria: - Major general or neurological comorbidity that is unrelated to HD. - Any other condition that in the opinion of the investigator warrants exclusion of the study. - Inability to understand the information about the study.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| - To investigate changes in employment (e.g. a job change, work cessation/early retirement, changes in working hours, and changes in duties/responsibilities) in HD, with a focus on improving the understanding of reasons behind work cessation. - To relate possible work related changes to psychiatric, behavioural, cognitive and motor (dys)function. Outcome measures questionnaires. - Work assessment: detailed demographics on employment and work cessation. This questionnaire is filled in by the participant as well as the proxy. - UBOS: overall total score and score per subscale; exhaustion, emotional distance and competence score. The test can be used to assess whether someone has a burn-out or not. This questionnaire is filled in by the participant as well as the proxy. - FrSBe: overall total score and total score per subscale; apathy, disinhibition, and executive dysfunction. This questionnaire is filled in by the participant as well as the proxy. - UCL: total scores per coping subscale; active and passive coping (7 * 28), palliation (8 * 32), avoidance (8 * 32), seeking support (6 * 24) , emotional expression (3 * 12) and seeking reassurance (5 * 20). This questionnaire is filled in by the participant as well as the proxy. - HADS-SIS: score per subscale; anxiety (0 * 21), depression (0 * 21), inward- and outward irritability (0 * 12). This questionnaire is filled in by the participant as well as the proxy. Outcome measures motor assessments - UHDRS-TMS: total motor score (0 * 124) - UHDRS-TFC: total functional capacity (0 * 13) Outcome measures psychiatric and behavioural evaluation. - PBA-s: total score per symptom is calculated by multiplying severity by frequency. Outcome measures neuropsychological assessment. - SDMT: total number of correct responses after 90 seconds. - Stroop: number of correct response after 45 seconds per trial. - TMT trial A and B: total seconds to complete the task. | — |
Secondary
| Measure | Time frame |
|---|---|
| - Determining whether burn out related symptoms are more prevalent in patients with HD than in the general population and ascertain in how many cases burn-out related symptoms can be marked as the onset of HD. - Report if work type and cultural background of participants influences employment and how this relates to cognitive, psychiatric and motor (dys)function. - Identifying whether limited self-insight influences work cessation by means of proxy measurement. - To contribute to better supply of information for patients, family, employers, health and safety officers and offices regarding employment and Huntingtons disease progression. - Identifying whether coping influences working capacity. For outcome measures see primary study parameters. | — |
Countries
Netherlands
Outcome results
None listed