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| Name | Class |
|---|---|
| European Huntington's Disease Network | NETWORK |
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The primary objective of this pilot study is to assess the feasibility of three internationally agreed pain test batteries in patients with Huntington Disease. The pain test batteries are developed to collect data about the facial expression of pain, body movements and vocalization of (non-) painful stimuli, and to collect data for testing the pain inhibition and facilitation. The study population will include 20 patients with genetically confirmed, adult-onset HD, in stage 1 (n=10) and stages 2 - 3 of HD (n = 10).
The primary objective of this pilot study is to assess the feasibility of a comprehensive experimental design with three internationally well-known experimental pain protocols in patients with Huntington Disease.
The exploratory objectives are:
Study design: An experimental, observational, cross-sectional study
Study population: The study population will include 20 patients with genetically confirmed, adult-onset HD, in stage 1 (n=10) and stages 2 - 3 of HD (n = 10).
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Adult-onset HD stage 1 | The adult-onset HD stage 1 group (total function capacity 11-13) include 10 participants. The subjects will be undergo three different pain test batteries . |
| |
| Adult-onset HD stage 2-3 | The adult-onset HD stage 2-3 group (total function capacity 3-10) include 10 participants. The subjects will be undergo three different pain test batteries . |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Facial expression block | Other | Data will be collected to assess the facial expression, body movements and vocalization of (non-) painful heat stimuli. The experiment includes individually tailored and randomly administered painful (perceived as moderate pain) and non-painful heat stimuli. |
| Measure | Description | Time Frame |
|---|---|---|
| The feasibility of a comprehensive experimental design including three different experimental pain protocols in patients with Huntington's Disease | A screening tool was developed to determine whether the comprehensive experimental design, with three various experimental pain protocols, is feasible in patients with HD. After each assessment, the screening tool will be scored. Each item of the screening tool represents an important part of the experimental pain protocol. A 'cut-off' score ranging from 60% to 80% was adopted for each item, indicating the minimum score to be met to consider as feasible for that specific part of the experimental pain protocol. The comprehension of the instructions, duration and the completion of the study are examples of items which will be evaluated. | Baseline assessment |
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| Measure | Description | Time Frame |
|---|---|---|
| Psychometric properties of the Pain Assessment in Impaired Cognition- 15 | The intra-, interrater, test-retest reliability and measurement error (i.e. agreement) of the facial expression, body movements and vocalization items of the Pain Assessment in Impaired Cognition - 15 will be assessed. The score of the Pain Assessment in Impaired Cognition - 15 ranges from 0 to 45 points. A score of 4 or higher indicate the presence of possible pain. |
Inclusion Criteria:
Exclusion Criteria:
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Genetically and clinically confirmed adult-onset HD patients (AoHD) (clinical onset ≥ 21 years, CAG repeats ≥ 36, Diagnostic Confidence Level [DCL] of 4]), in stage 1 (n=10 [named as HD 1]), stage 2 and 3 of HD (n = 10 [named as HD 2]), according to the Shoulson-Fahn system. Total Function Capacity (TFC) of the Unified Huntington Disease Rating Scale (UHDRS) will be used to define the HD groups: UHDRS-TFC score 11-13 (stage 1), 7-10 (stage 2), 3-6 (stage 3), 1-2 (stage 4) and a score of 0 (stage 5).
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Gregory Sprenger, Msc | Contact | +31 71 526 54 42 | g.p.sprenger@lumc.nl | |
| Susanne de Bot, MD, Phd | Contact | +31 71 5262197 | huntingtononderzoek@lumc.nl |
| Name | Affiliation | Role |
|---|---|---|
| Susanne de Bot, MD Phd | Leiden University Medical Center | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Leiden University Medical Center | Leiden | South Holland | 2333 ZA | Netherlands |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 22480803 | Background | Yarnitsky D, Granot M, Nahman-Averbuch H, Khamaisi M, Granovsky Y. Conditioned pain modulation predicts duloxetine efficacy in painful diabetic neuropathy. Pain. 2012 Jun;153(6):1193-1198. doi: 10.1016/j.pain.2012.02.021. Epub 2012 Apr 3. | |
| 25330039 | Background | Yarnitsky D, Bouhassira D, Drewes AM, Fillingim RB, Granot M, Hansson P, Landau R, Marchand S, Matre D, Nilsen KB, Stubhaug A, Treede RD, Wilder-Smith OH. Recommendations on practice of conditioned pain modulation (CPM) testing. Eur J Pain. 2015 Jul;19(6):805-6. doi: 10.1002/ejp.605. Epub 2014 Oct 20. |
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Aim is to comply with the standards of the Leiden University Medical Center (LUMC) and the contract of the funder of the study: European Huntington Disease Network (EHDN)
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| ID | Term |
|---|---|
| D006816 | Huntington Disease |
| D010146 | Pain |
| ID | Term |
|---|---|
| D001480 | Basal Ganglia Diseases |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
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|
| Conditioned pain modulation block | Other | The conditioned pain modulation will be tested according to internationally agreed standards and includes a painful phasic heat (test stimulus) and a painful tonic cold stimulus (conditioning stimulus). |
|
| Pain facilitation block | Other | Pain facilitation will be assessed by using painful mechanical pinprick stimuli. The magnitude of pain experienced after a single mechanical pinprick stimulus is compared to that of a train of 10 pinprick stimuli of the same force. |
|
| Baseline assessment - 15 minutes |
| Overall facial expression of pain in patients with HD | The facial expression will be assessed with the Pain Assessment in Impaired Cognition Scale. The score on the Pain Assessment in Impaired Cognition Scale ranges from 0 to 45 points. A score of 4 or higher indicate the presence of possible pain. | Baseline assessment - 15 minutes |
| Conditioned pain modulation | Pain processing in terms of conditioned pain modulation( pain facilitation, inhibition and the balance between two) will be assessed by the (Computerized) Visual Analogue Scale (CoVAS). The (Computerized) Visual Analogue Scale is a straight line with one end meaning no pain and the other end meaning the worst pain imaginable. A subject marks a point of the line that matches the amount of pain he or she feels. | Baseline assessment - 15 minutes |
| Conditioned pain modulation | Pain processing in terms of conditioned pain modulation( pain facilitation, inhibition and the balance between two) will be assessed by the numerical rating scale (NRS) in case the Computerized Visual Analogue Scale is not feasible to use. The numerical rating scale consists of a series of numbers rating pain intensity, ranging from 0 to 10, with being 0 being "no pain", and 10 "severe pain". | Baseline assessment - 15 minutes |
| Measurement error Condition Pain Modulation | An initial estimate of the measurement error (i.e. agreement) will be tested for the outcome of the endogenous pain modulation. The (Computerized) Visual Analogue Scale is a straight line with one end meaning no pain and the other end meaning the worst pain imaginable. A subject marks a point of the line that matches the amount of pain he or she feels. An appropriate analysis will conducted such as (weighted kappa), Intraclass correlation coefficient, SEM and/ or Limits of agreement. | Area under the curve; with a time frame 0,1,2,3,4,5,6,7,8,9 to 10 seconds. |
| Measurement error Condition Pain Modulation | An initial estimate of the measurement error (i.e. agreement) will be tested for the outcome of the endogenous pain modulation. The numerical rating scale consists of a series of numbers rating pain intensity, ranging from 0 to 10, with being 0 being "no pain", and 10 "severe pain". An appropriate analysis will conducted such as (weighted kappa), Intraclass correlation coefficient, SEM and/ or Limits of agreement. | Difference in mean score of the Numerical Rating Scale. Numerical Rating Scale is scored directly after the stimulus. The duration of the exposure is 10seconds. |
| 18783885 | Background | Kunz M, Chatelle C, Lautenbacher S, Rainville P. The relation between catastrophizing and facial responsiveness to pain. Pain. 2008 Nov 15;140(1):127-134. doi: 10.1016/j.pain.2008.07.019. Epub 2008 Sep 9. |
| 31487411 | Background | Kunz M, de Waal MWM, Achterberg WP, Gimenez-Llort L, Lobbezoo F, Sampson EL, van Dalen-Kok AH, Defrin R, Invitto S, Konstantinovic L, Oosterman J, Petrini L, van der Steen JT, Strand LI, de Tommaso M, Zwakhalen S, Husebo BS, Lautenbacher S. The Pain Assessment in Impaired Cognition scale (PAIC15): A multidisciplinary and international approach to develop and test a meta-tool for pain assessment in impaired cognition, especially dementia. Eur J Pain. 2020 Jan;24(1):192-208. doi: 10.1002/ejp.1477. Epub 2019 Oct 13. |
| 31520424 | Background | de Waal MWM, van Dalen-Kok AH, de Vet HCW, Gimenez-Llort L, Konstantinovic L, de Tommaso M, Fischer T, Lukas A, Kunz M, Lautenbacher S, Lobbezoo F, McGuire BE, van der Steen JT, Achterberg WP. Observational pain assessment in older persons with dementia in four countries: Observer agreement of items and factor structure of the Pain Assessment in Impaired Cognition. Eur J Pain. 2020 Feb;24(2):279-296. doi: 10.1002/ejp.1484. Epub 2019 Oct 22. |
| 41961860 | Derived | Sprenger GP, van Velzen M, Achterberg WP, Roos RAC, de Bot ST. Pain processing and pain assessment in Huntington's disease: Study protocol of the Huntington's disease - PAIN study. PLoS One. 2026 Apr 10;21(4):e0346039. doi: 10.1371/journal.pone.0346039. eCollection 2026. |
| 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 |
| D009461 | Neurologic Manifestations |
| D012816 | Signs and Symptoms |
| D013568 | Pathological Conditions, Signs and Symptoms |