None listed
Conditions
Brief summary
Huntington's Disease is a disabling neurodegenerative disorder with no effective therapy. In addition to a known genetic defect, Huntington's is characterized by major defects in cell energy production, which may be partly or primarily responsible for degeneration and death of neurons. Ketogenic diets are high-fat, adequate-protein, low-carbohydrate diets that produce molecules called ketones, which have many energy advantages over glucose as a brain fuel and may help the symptoms. We aim to perform a study in 5-10 people with Huntington’s to examine whether 12 weeks of a ketogenic diet is feasible and improves symptoms in this challenging disorder.
Interventions
A modified ketogenic diet. The modified ketogenic plan will be based on recipes containing vegetables, fish, poultry, coconut-based products, avocados, nuts, and natural oils; it will be high in fat (60-65% of energy intake, by weight), moderate in protein (25-30%), and low in carbohydrate (10-15%). There will be space to record daily (bedtime) blood glucose and ketone levels.
The lead investigator and nutrition specialists will show participants how to follow the diet plan. The plan will be a
A modified ketogenic diet. The modified ketogenic plan will be based on recipes containing vegetables, fish, poultry, coconut-based products, avocados, nuts, and natural oils; it will be high in fat (60-65% of energy intake, by weight), moderate in protein (25-30%), and low in carbohydrate (10-15%). There will be space to record daily (bedtime) blood glucose and ketone levels. The lead investigator and nutrition specialists will show participants how to follow the diet plan. The plan will be adhered to daily for 12 weeks. Participants will do this intervention in their own home. Regular support and education sessions will be provided: - Single 40-minute group education session at screening visit discussing content related to concept and potential adverse effects of a keto diet. - Two 10-minute phone calls during the study regarding content on patient experiences with the keto diet (one in week 1, one in week 7). - The lead investigator and nutrition specialist will send global e-mails every 2-3 days and we may film and post 10-minute videos on a study website, content answering questions from participants and providing expectations on what to expect with keto diet. - Single food diary at the start of intervention regarding patient usual diet, adherence will be monitored daily throughout the study using blood glucose and ketone monitor recordings as this is a superior method to ensure ketosis (ie it is a direct measure of ketosis) and food diaries are burdensome for patients.
Sponsors
Waikato Hospital
Study design
Allocation
Non-randomised trial
Intervention model
Single group
Primary purpose
Treatment
Masking
Open (masking not used)
Eligibility
Sex/Gender
All
Age
20 Years to 80 Years
Healthy volunteers
No
Inclusion criteria
(1) Aged 20 to 80 years. (2) Huntington’s confirmed by genetic diagnosis with 40 or more CAG repeats and definite motor manifestation as determined by a neurologist. (3) Sufficient consent capacity (passing grade on the modified dementia Informed Consent questionnaire, defined as at least 70% of questions 1 to 10 correct and 100% of questions 11 to 14 correct on the first or second administration). (4) BMI >18.5. (5) A cohabiting study partner able and willing to follow the diet plan.
Exclusion criteria
(1) Inability to speak or understand English. (2) A concurrent medical or psychiatric condition that in the opinion of the investigators would make it difficult to complete the study.
Outcome results
None listed