A Pilot Study of Nutritional Status in Patients With Huntington's Disease

Study Overview
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Brief Summary
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It is known that weight loss is a poor prognostic marker in HD, but it is not known which dietary interventions are optimal at different stages of the disease. Current guidelines for HD treatment are informed only by studies in people with other causes of weight loss. Our long term goal is to create the evidence base for improved nutritional management in HD. This study will pilot the tools to inform the development of clinical trials protocols. We want to know which measures can be used by patients manifesting obvious clinical features of HD, and which are most helpful in detecting clinically meaningful changes in nutrition status.

Detailed Description
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Huntington's disease (HD) is a complex autosomal dominant disorder of (generally) adult onset. The clinical features of HD are cognitive decline, psychiatric disturbance, personality change and movement disorder. Although by EU definition a rare disease, better management and establishment of clinics for care of HD around the world have led to recognition of that the condition is much more common than previously considered. Grampian has one of the longest established clinics for managing HD worldwide, and we now care for more than 150 people at different stages in HD, making us one of the largest five specialist clinics in the UK. Members of our team have led the production of European guidelines for HD management, and within this project, we have highlighted the importance of nutrition in HD. Weight loss is well documented as a clinical feature of all stages of Huntington's disease (HD). However, its metabolic basis is poorly understood and appetite has not been formally studied in patients with the condition. Furthermore, the composition of weight loss - muscle or fat has been little studied and modern nutrition assessment tools have not been applied in this cohort. It is known that weight loss is a poor prognostic marker in HD, but it is not known which dietary interventions are optimal at different stages of the disease. Current guidelines for HD treatment are informed only by studies in people with other causes of weight loss. The long term goal of this study is to create the evidence base for improved nutritional management in HD. This study will pilot the tools to inform the development of clinical trials protocols. The aim is to identify which measures can be used by patients manifesting obvious clinical features of HD, and which are most helpful in detecting clinically meaningful changes in nutrition status.

Official Title
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A Pilot Study of Nutritional Status in Patients With Huntington's Disease

Conditions
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Huntington Disease

Intervention / Treatment
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* Diagnostic Test: Body composition using a hand-held body fat monitor • Resting Metabolic Rate (RMR) using a ventilated hood system


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: stage 2 or 3 Huntington disease Exclusion Criteria: unable to give consent

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| weight stable<br>stage 2 and 3 as defined by the UHDRS Total Functional Capacity score will be recruited. Ten will have reported at least 5% weight loss in a 12-month period, | Diagnostic Test: Body composition using a hand-held body fat monitor • Resting Metabolic Rate (RMR) using a ventilated hood system<br>* Consent Paperwork Nutritional assessment using MNA and MUST questionnaires 24-hour dietary recall assessment Body weight, height, waist circumference, mid upper arm and calf circumference<br>* Other names: Nutritional assessment;|
| weight loss<br>stage 2 and 3 as defined by the UHDRS Total Functional Capacity score will be recruited. Ten will have reported at least 5% weight loss in a 12-month period, | Diagnostic Test: Body composition using a hand-held body fat monitor • Resting Metabolic Rate (RMR) using a ventilated hood system<br>* Consent Paperwork Nutritional assessment using MNA and MUST questionnaires 24-hour dietary recall assessment Body weight, height, waist circumference, mid upper arm and calf circumference<br>* Other names: Nutritional assessment;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| satisfactory completion of body composition | measurement of body composition using hand-held body fat monitor | assessed at study visit for each participant | 
| measurement of resting metabolic rate | measurement of Resting Metabolic Rate (RMR) using a ventilated hood system | assessed at study visit for each participant | 
| measurement of body weight | body weight in kg | assessed at study visit for each participant | 
| measurement of height | height in metres | assessed at study visit for each participant | 
| completion of measurement of waist, arm and calf circumference | measurement of waist circumference, mid upper arm and calf circumference using a tape measure | assessed at study visit for each participant | 
| nutritional assessment | estimation of energy, protein, carbohydrate and fat intake using MNA and MUST questionnaires | assessed at study visit for each participant | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| variability of measures to power future grant applications | Descriptive statistics of each measurement by group | at end of study |