Huntington's Disease Progression: A Population Modeling Approach to Characterization Using Clinical Rating Scales

2020 ◽  
Vol 60 (8) ◽  
pp. 1051-1060
Author(s):  
Wan Sun ◽  
Di Zhou ◽  
John H. Warner ◽  
Douglas R. Langbehn ◽  
Guenther Hochhaus ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Progression ◽  
Rating Scales ◽  
Population Modeling ◽  
Modeling Approach ◽  
Clinical Rating

scholarly journals Exposure of R6/2 mice in an enriched environment augments P42 therapy efficacy on Huntington's disease progression

2021 ◽  
Vol 186 ◽  
pp. 108467
Author(s):  
Simon Couly ◽  
Allison Carles ◽  
Morgane Denus ◽  
Lorraine Benigno-Anton ◽  
Florence Maschat ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Progression ◽  
Enriched Environment ◽  
Therapy Efficacy

scholarly journals Circadian-based Treatment Strategy Effective in the BACHD Mouse Model of Huntington’s Disease

2018 ◽  
Vol 33 (5) ◽  
pp. 535-554 ◽  
Author(s):  
Daniel S. Whittaker ◽  
Dawn H. Loh ◽  
Huei-Bin Wang ◽  
Yu Tahara ◽  
Dika Kuljis ◽  
...  
Keyword(s):  
Mouse Model ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Progression ◽  
Sleep Onset ◽  
Lifestyle Changes ◽  
Disease Stage ◽  
Preclinical Model ◽  
Delay Disease Progression ◽  
Circadian Dysfunction

Huntington’s disease (HD) patients suffer from progressive neurodegeneration that results in cognitive, psychiatric, cardiovascular, and motor dysfunction. Disturbances in sleep-wake cycles are common among HD patients with reports of delayed sleep onset, frequent bedtime awakenings, and excessive fatigue. The BACHD mouse model exhibits many HD core symptoms including circadian dysfunction. Because circadian dysfunction manifests early in the disease in both patients and mouse models, we sought to determine if early interventions that improve circadian rhythmicity could benefit HD symptoms and delay disease progression. We evaluated the effects of time-restricted feeding (TRF) on the BACHD mouse model. At 3 months of age, the animals were divided into 2 groups: ad lib and TRF. The TRF-treated BACHD mice were exposed to a 6-h feeding/18-h fasting regimen that was designed to be aligned with the middle (ZT 15-21) of the period when mice are normally active (ZT 12-24). Following 3 months of treatment (when mice reached the early disease stage), the TRF-treated BACHD mice showed improvements in their locomotor activity and sleep behavioral rhythms. Furthermore, we found improved heart rate variability, suggesting that their autonomic nervous system dysfunction was improved. On a molecular level, TRF altered the phase but not the amplitude of the PER2::LUC rhythms measured in vivo and in vitro. Importantly, treated BACHD mice exhibited improved motor performance compared with untreated BACHD controls, and the motor improvements were correlated with improved circadian output. It is worth emphasizing that HD is a genetically caused disease with no known cure. Lifestyle changes that not only improve the quality of life but also delay disease progression for HD patients are greatly needed. Our study demonstrates the therapeutic potential of circadian-based treatment strategies in a preclinical model of HD.

Intraventricular Administration of a Caspase Inhibitor Delays Disease Progression and Mortality in a Mouse Model of Huntington's Disease

Neurosurgery ◽  
1999 ◽  
Vol 45 (3) ◽  
pp. 718-718 ◽  
Author(s):  
Robert Friedlander ◽  
Victor O. Ona ◽  
Mingwei Li ◽  
Jean Paul G. Vonsattel ◽  
L. John Andrews ◽  
...  
Keyword(s):  
Mouse Model ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Progression ◽  
Intraventricular Administration ◽  
Caspase Inhibitor

Rating scales for cognition in Huntington's disease: Critique and recommendations

2017 ◽  
Vol 33 (2) ◽  
pp. 187-195 ◽  
Author(s):  
Tiago A. Mestre ◽  
Anne-Catherine Bachoud-Lévi ◽  
Johan Marinus ◽  
Julie C. Stout ◽  
Jane S. Paulsen ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scales

scholarly journals The relationship between uric acid levels and Huntington's disease progression

2010 ◽  
Vol 25 (2) ◽  
pp. 224-228 ◽  
Author(s):  
Peggy Auinger ◽  
Karl Kieburtz ◽  
Michael P. Mcdermott
Keyword(s):  
Uric Acid ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Progression ◽  
The Relationship

GENETIC MODIFIERS OF HUNTINGTON'S DISEASE PROGRESSION

2016 ◽  
Vol 87 (12) ◽  
pp. e1.35-e1
Author(s):  
Davina Hensman Moss ◽  
Antonio Pardiñas ◽  
Michael Flower ◽  
James Miller ◽  
Kitty Lo ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Progression ◽  
Genetic Modifiers

Environmental enrichment slows disease progression in R6/2 Huntington's disease mice

2002 ◽  
Vol 51 (2) ◽  
pp. 235-242 ◽  
Author(s):  
Emma Hockly ◽  
Patricia M. Cordery ◽  
Benjamin Woodman ◽  
Amarbirpal Mahal ◽  
Anton Van Dellen ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Progression ◽  
Environmental Enrichment
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