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

• Conditions: Huntington Disease
• Interventions: Diagnostic Test: Body composition using a hand-held body fat monitor • Resting Metabolic Rate (RMR) using a ventilated hood system

• Registration Number: NCT05668520
• Lead Sponsor: University of Aberdeen

Brief Summary

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

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.

Study Timeline

• Study First Submitted: 2022-09-30
• Study Start: 2022-10-01
• Status Verified: 2022-12
• Study First Submitted QC: 2022-12-20
• Last Update Submitted: 2022-12-20
• Study First Posted: 2022-12-29
• Last Update Posted: 2022-12-29
• Primary Completion: 2023-10
• Study Completion: 2023-10

Recruitment & Eligibility

• Status: ENROLLING_BY_INVITATION
• Sex: All
• Target Recruitment: 20

Inclusion Criteria

• stage 2 or 3 Huntington disease

Exclusion Criteria

• unable to give consent

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
weight loss	Body composition using a hand-held body fat monitor • Resting Metabolic Rate (RMR) using a ventilated hood system	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,
weight stable	Body composition using a hand-held body fat monitor • Resting Metabolic Rate (RMR) using a ventilated hood system	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,

Primary Outcome Measures

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

Secondary Outcome Measures

Name	Time	Method
variability of measures to power future grant applications	at end of study	Descriptive statistics of each measurement by group

Trial Locations

Locations (1)

Zosia Miedzybrodzka; 🇬🇧 Aberdeen, Scotland, United Kingdom