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Time-Restricted Ketogenic Diet in Huntington's Disease: A Case Study

Huntington's disease (HD) is a progressive, fatal neurodegenerative disorder with limited treatment options. Substantial evidence implicates mitochondria dysfunction in brain and skeletal muscle in the pathogenesis of HD. Metabolic strategies, such as fasting and ketogenic diets, theoretically...

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Autores principales: Phillips, Matthew C. L., McManus, Eileen J., Brinkhuis, Martijn, Romero-Ferrando, Beatriz
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9372583/
https://www.ncbi.nlm.nih.gov/pubmed/35967897
http://dx.doi.org/10.3389/fnbeh.2022.931636
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author Phillips, Matthew C. L.
McManus, Eileen J.
Brinkhuis, Martijn
Romero-Ferrando, Beatriz
author_facet Phillips, Matthew C. L.
McManus, Eileen J.
Brinkhuis, Martijn
Romero-Ferrando, Beatriz
author_sort Phillips, Matthew C. L.
collection PubMed
description Huntington's disease (HD) is a progressive, fatal neurodegenerative disorder with limited treatment options. Substantial evidence implicates mitochondria dysfunction in brain and skeletal muscle in the pathogenesis of HD. Metabolic strategies, such as fasting and ketogenic diets, theoretically enhance brain and muscle metabolism and mitochondria function, which may improve the clinical symptoms of HD. We report the case of a 41-year-old man with progressive, deteriorating HD who pursued a time-restricted ketogenic diet (TRKD) for 48 weeks. Improvements were measured in his motor symptoms (52% improvement from baseline), activities of daily living (28% improvement), composite Unified HD Rating Scale (cUHDRS) score (20% improvement), HD-related behavior problems (apathy, disorientation, anger, and irritability improved by 50–100%), and mood-related quality of life (25% improvement). Cognition did not improve. Weight remained stable and there were no significant adverse effects. This case study is unique in that a patient with progressive, deteriorating HD was managed with a TRKD, with subsequent improvements in his motor symptoms, activities of daily living, cUHDRS score, most major HD-related behavior problems, and quality of life. Our patient remains dedicated to his TRKD, which continues to provide benefit for him and his family.
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spelling pubmed-93725832022-08-13 Time-Restricted Ketogenic Diet in Huntington's Disease: A Case Study Phillips, Matthew C. L. McManus, Eileen J. Brinkhuis, Martijn Romero-Ferrando, Beatriz Front Behav Neurosci Behavioral Neuroscience Huntington's disease (HD) is a progressive, fatal neurodegenerative disorder with limited treatment options. Substantial evidence implicates mitochondria dysfunction in brain and skeletal muscle in the pathogenesis of HD. Metabolic strategies, such as fasting and ketogenic diets, theoretically enhance brain and muscle metabolism and mitochondria function, which may improve the clinical symptoms of HD. We report the case of a 41-year-old man with progressive, deteriorating HD who pursued a time-restricted ketogenic diet (TRKD) for 48 weeks. Improvements were measured in his motor symptoms (52% improvement from baseline), activities of daily living (28% improvement), composite Unified HD Rating Scale (cUHDRS) score (20% improvement), HD-related behavior problems (apathy, disorientation, anger, and irritability improved by 50–100%), and mood-related quality of life (25% improvement). Cognition did not improve. Weight remained stable and there were no significant adverse effects. This case study is unique in that a patient with progressive, deteriorating HD was managed with a TRKD, with subsequent improvements in his motor symptoms, activities of daily living, cUHDRS score, most major HD-related behavior problems, and quality of life. Our patient remains dedicated to his TRKD, which continues to provide benefit for him and his family. Frontiers Media S.A. 2022-07-29 /pmc/articles/PMC9372583/ /pubmed/35967897 http://dx.doi.org/10.3389/fnbeh.2022.931636 Text en Copyright © 2022 Phillips, McManus, Brinkhuis and Romero-Ferrando. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Behavioral Neuroscience
Phillips, Matthew C. L.
McManus, Eileen J.
Brinkhuis, Martijn
Romero-Ferrando, Beatriz
Time-Restricted Ketogenic Diet in Huntington's Disease: A Case Study
title Time-Restricted Ketogenic Diet in Huntington's Disease: A Case Study
title_full Time-Restricted Ketogenic Diet in Huntington's Disease: A Case Study
title_fullStr Time-Restricted Ketogenic Diet in Huntington's Disease: A Case Study
title_full_unstemmed Time-Restricted Ketogenic Diet in Huntington's Disease: A Case Study
title_short Time-Restricted Ketogenic Diet in Huntington's Disease: A Case Study
title_sort time-restricted ketogenic diet in huntington's disease: a case study
topic Behavioral Neuroscience
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9372583/
https://www.ncbi.nlm.nih.gov/pubmed/35967897
http://dx.doi.org/10.3389/fnbeh.2022.931636
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