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

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.

Study Overview

• Status: Enrolling by invitation
• Conditions: Huntington Disease
• Intervention / Treatment: Diagnostic test: Body composition using a hand-held body fat monitor • Resting Metabolic Rate (RMR) using a ventilated hood system

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 Type: Observational
• Enrollment (Anticipated): 20

Contacts and Locations

Study Locations

• United Kingdom
  • Scotland
    • Aberdeen, Scotland, United Kingdom, AB25 2ZA
      • Zosia Miedzybrodzka

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

manifesting Huntington disease patients of North of Scotland Huntington's clinic

Description

Inclusion Criteria:

• stage 2 or 3 Huntington disease

Exclusion Criteria:

• unable to give consent

Study Plan

How is the study designed?

Design Details

• Observational Models: Case-Only
• Time Perspectives: Prospective

Number of groups / cohorts

2

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
weight stable; 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; 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; Other Names: Nutritional assessment
weight loss; 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; 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; Other Names: Nutritional assessment

What is the study measuring?

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

Collaborators and Investigators

• Sponsor: University of Aberdeen

Study record dates

Study Major Dates

• Study Start (Actual): October 1, 2022
• Primary Completion (Anticipated): October 1, 2023
• Study Completion (Anticipated): October 1, 2023

Study Registration Dates

• First Submitted: September 30, 2022
• First Submitted That Met QC Criteria: December 20, 2022
• First Posted (Estimate): December 29, 2022

Study Record Updates

• Last Update Posted (Estimate): December 29, 2022
• Last Update Submitted That Met QC Criteria: December 20, 2022
• Last Verified: December 1, 2022

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Mental Disorders; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Neurocognitive Disorders; Genetic Diseases, Inborn; Basal Ganglia Diseases; Movement Disorders; Neurodegenerative Diseases; Dyskinesias; Heredodegenerative Disorders, Nervous System; Dementia; Cognition Disorders; Chorea; Huntington Disease
• Other Study ID Numbers: 2-095-19

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: Undecided
• IPD Plan Description: de-identified data sharing would be considered on request but would require additional permissions

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No