Huntington's Disease and Pain

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:

* To explore the psychometric properties (inter-, intra-rater and test-retest reliability and measurement error) of the facial expression, body movements and vocalization items of an observational pain scale, the Pain Assessment in Impaired Cognition Scale (PAIC15), in patients with HD.
* To measure the overall facial expression of pain in patients with HD.
* To explore the prevalence and extent of endogenous pain modulation in HD (facilitation, inhibition and the balance between the two).
* To make an initial estimate of the measurement error (i.e. agreement), stated as the systematic and random error of a patients' score that is not attributed to true changes in the construct to be measured, for each endpoint related in current study to pain processing in HD (e.g. facial expression and the endogenous pain modulation outcomes).

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).

Eligibility Criteria:
Inclusion Criteria:

1. Genetically and clinically confirmed Adult-onset HD patients (≥ 21 years, CAG repeats ≥ 36; DCL of 4).
2. Good general health apart from having HD. Note: Patients with chronic illness (e.g. hypertension) will be eligible if the illness is stable and well-controlled according to the investigator that will not impact the primary objectives of the study.
3. Able to give written informed consent

Exclusion Criteria:

1. Juvenile and Pediatric Huntington's Disease (age at onset \<21 years).
2. Patients in the late stage of the disease (UDHRS-TFC score \<3).
3. Have medical, psychiatric, or other conditions (other than HD) that, according to the investigator, may compromise the patient's ability to understand the patient information sheet, to give informed consent, to comply with all study requirements, or to perform study assessments.
4. Have a history of (in the past year) or current (ab)use of any drug, alcohol or medication that, in the opinion of the investigator, may seriously interfere with the primary objectives of the study.
5. The presence of a sensorimotor neuropathy or any another disturbance significantly disturbing the somatosensory systems, based on medical history and/or clinical examination, that can interfere with the pain test battery.
6. Women who are pregnant or breastfeeding