scholarly journals Thalamic Atrophy in Huntington's Disease Co-varies with Cognitive Performance: A Morphometric MRI Analysis

2004 ◽  
Vol 15 (6) ◽  
pp. 846-853 ◽  
Author(s):  
Jan Kassubek ◽  
Freimut D. Juengling ◽  
Daniel Ecker ◽  
G. Bernhard Landwehrmeyer
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Performance

scholarly journals Visual scanning and cognitive performance in prediagnostic and early-stage Huntington's disease

2009 ◽  
Vol 24 (4) ◽  
pp. 533-540 ◽  
Author(s):  
Tanya Blekher ◽  
Marjorie R. Weaver ◽  
Jeanine Marshall ◽  
Siu Hui ◽  
Jacqueline Gray Jackson ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Performance ◽  
Early Stage ◽  
Visual Scanning

scholarly journals Gut dysbiosis in Huntington’s disease: associations among gut microbiota, cognitive performance and clinical outcomes

2020 ◽  
Vol 2 (2) ◽  
Author(s):  
Cory I Wasser ◽  
Emily-Clare Mercieca ◽  
Geraldine Kong ◽  
Anthony J Hannan ◽  
Sonja J McKeown ◽  
...  
Keyword(s):  
Gut Microbiota ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Progression ◽  
Cognitive Performance ◽  
Gut Microbiome ◽  
Disease Gene ◽  
Healthy Controls ◽  
Gut Dysbiosis ◽  
Gene Expansion

Abstract Huntington’s disease is characterized by a triad of motor, cognitive and psychiatric impairments, as well as unintended weight loss. Although much of the research has focused on cognitive, motor and psychiatric symptoms, the extent of peripheral pathology and the relationship between these factors, and the core symptoms of Huntington’s disease, are relatively unknown. Gut microbiota are key modulators of communication between the brain and gut, and alterations in microbiota composition (dysbiosis) can negatively affect cognition, behaviour and affective function, and may be implicated in disease progression. Furthermore, gut dysbiosis was recently reported in Huntington’s disease transgenic mice. Our main objective was to characterize the gut microbiome in people with Huntington’s disease and determine whether the composition of gut microbiota are significantly related to clinical indicators of disease progression. We compared 42 Huntington’s disease gene expansion carriers, including 19 people who were diagnosed with Huntington’s disease (Total Functional Capacity > 6) and 23 in the premanifest stage, with 36 age- and gender-matched healthy controls. Participants were characterized clinically using a battery of cognitive tests and using results from 16S V3 to V4 rRNA sequencing of faecal samples to characterize the gut microbiome. For gut microbiome measures, we found significant differences in the microbial communities (beta diversity) based on unweighted UniFrac distance (P = 0.001), as well as significantly lower alpha diversity (species richness and evenness) between our combined Huntington’s disease gene expansion carrier group and healthy controls (P = 0.001). We also found major shifts in the microbial community structure at Phylum and Family levels, and identified functional pathways and enzymes affected in our Huntington’s disease gene expansion carrier group. Within the Huntington’s disease gene expansion carrier group, we also discovered associations among gut bacteria, cognitive performance and clinical outcomes. Overall, our findings suggest an altered gut microbiome in Huntington’s disease gene expansion carriers. These results highlight the importance of gut biomarkers and raise interesting questions regarding the role of the gut in Huntington’s disease, and whether it may be a potential target for future therapeutic intervention.

scholarly journals Validation Study of a German Cognitive Battery for Huntington’s Disease: Relationship Between Cognitive Performance, Functional Decline, and Disease Burden

2020 ◽  
Author(s):  
Alžbeta Mühlbäck ◽  
Wiebke Frank ◽  
Olga Klempířová ◽  
Ondřej Bezdíček ◽  
Lena Schmitt ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Diagnostic Accuracy ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Performance ◽  
Disease Burden ◽  
Disease Duration ◽  
Characteristic Curve ◽  
Functional Decline ◽  
Color Naming

Abstract Objective Cognitive decline is a key characteristic of Huntington’s disease (HD). This study aimed to investigate the diagnostic accuracy of a cognitive battery with six tests used by most HD research centers to assess cognitive impairment in HD. Method In total, 106 HD patients in different disease stages with more (HD-CD, N = 30) and less cognitive impairments (HD-NC, N = 70) and 100 healthy controls (NC) were matched by age, sex, and education and were examined using a standardized protocol including cognitive, motor, and functional assessments. Results One-way between-groups analysis of variance showed that controls performed significantly better than HD patients and that HD-NC significantly outperformed HD-CD patients in all cognitive tests (NC > HD-NC > HD-CD), with all Games-Howell post-hoc tests p < .001. Analyses using area under the receiver-operating characteristic curve (AUC) disclosed the diagnostic accuracy of all tests included in the battery to discriminate between NC and HD patients with AUC ranging from 0.809 to 0.862 (all p < .001) and between HD-CD and HD-NC patients with AUC ranging from 0.833 to 0.899 (all p < .001). In both analysis, Stroop Color Naming Test showed the highest discriminative potential. Additional analyses showed that cognitive deficits in all domains progressed with disease duration. Moreover, cognitive performance correlated with the severity of motor and functional impairment (all p < .001) and with the Disease Burden Score regardless of disease duration and age. Conclusion Our results indicate that the cognitive battery is a suitable tool for assessing cognitive impairment in HD.

scholarly journals Metacognitive insight into cognitive performance in pre and early-stage Huntington's disease

2021 ◽  
Author(s):  
Samuel RC Hewitt ◽  
Alice J White ◽  
Sarah L Mason ◽  
Roger A Barker
Keyword(s):  
Quality Of Life ◽  
Decision Making ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Performance ◽  
Disease Gene ◽  
Disease Stage ◽  
Gene Carriers ◽  
Insight Into

ABSTRACT Objectives Insight is an important predictor of quality of life in Huntington's disease and other neurodegenerative conditions. However, estimating insight with traditional methods such as questionnaires is challenging and subject to limitations. This study experimentally quantified metacognitive insight into cognitive performance in Huntington's disease gene-carriers. Methods We dissociated perceptual decision-making performance and metacognitive insight into performance in healthy controls (n=29), premanifest (n=19) and early-manifest (n=10) Huntington's disease gene-carriers. Insight was operationalised as the degree to which a participant's confidence in their performance was informative of their actual performance (metacognitive efficiency) and estimated using a computational model (HMeta-d). Results We found that pre and early-manifest Huntington's disease gene-carriers were impaired in making perceptual decisions compared to controls. Gene-carriers required more evidence in favour of the correct choice to achieve similar performance and perceptual impairments were increased in those with manifest disease. Surprisingly, despite marked perceptual impairments, Huntington's disease gene-carriers retained metacognitive insight into their perceptual performance. This was the case after controlling for confounding variables and regardless of disease stage. Conclusion We report for the first time a dissociation between impaired cognition and intact metacognition (trial-by-trial insight) in the early-stages of a neurodegenerative disease. This unexpected finding contrasts with the prevailing assumption that cognitive deficits are associated with impaired insight. Future studies should investigate how intact metacognitive insight could be used by some early Huntington's disease gene-carriers to positively impact their quality of life. Key words: Huntington's disease, decision-making, cognition, insight, metacognition

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