Pilot study of nutritional status in patients with Huntington disease
Research type
Research Study
Full title
A pilot study of nutritional status in patients with Huntington’s disease
IRAS ID
271336
Contact name
Zosia Miedzybrodzka
Contact email
Sponsor organisation
Ms Louise King
Duration of Study in the UK
2 years, 4 months, 1 days
Research summary
Huntington’s disease (HD) is a complex autosomal dominant disorder of (generally) adult onset. The cardinal 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 pattern of weight loss – muscle or fat has been little studied and modern nutrition assessment tools have not been used.
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.REC name
North of Scotland Research Ethics Committee 2
REC reference
20/NS/0023
Date of REC Opinion
14 Feb 2020
REC opinion
Favourable Opinion