scholarly journals Evaluation of gait initiation parameters using inertial sensors in Huntington's Disease: Insights into anticipatory postural adjustments and cognitive interference

2020 ◽  
Author(s):  
Radhika Desai ◽  
Nora E Fritz ◽  
Lisa Muratori ◽  
Jeffrey M. Hausdorff ◽  
Lori Quinn
Keyword(s):  
Cognitive Load ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Gait Speed ◽  
Load Condition ◽  
Anticipatory Postural Adjustments ◽  
Gait Initiation ◽  
Healthy Controls ◽  
Postural Adjustments ◽  
Single Task

Background and Purpose: Understanding the contribution of anticipatory postural adjustments (APA) on walking ability in individuals with Huntington's disease (HD) may provide insight into motor planning and the functional consequences of HD-specific cortical-basal ganglia pathway dysfunctions. The purpose of this study was to evaluate inertial measurement unit (IMU)-derived measures of APAs and first step parameters, and their contribution to gait speed, in individuals with and without manifest HD during a single-task and cognitive load condition. Methods: 33 individuals with manifest HD and 15 age-matched healthy controls wore three Opal APDM IMUs during a 14-meter walk during a single task and cognitive load condition. APA acceleration amplitudes, APA durations, first step range of motion (ROMs), and first step duration were compared, along with their relationship to gait speed. Results: Individuals with HD had significantly greater APA acceleration amplitudes, smaller first step ROMs and longer first step durations compared to healthy controls. No difference in APA durations were present between groups across conditions. Linear model results and significant correlations between mediolateral APA acceleration amplitudes and APA durations were found. Conclusions: Larger acceleration amplitudes, smaller first step ROMs of greater duration, accompanied by the preservation of APA durations reveal a discrepancy in movement scaling in HD. Additionally, the mediolateral component of the APA is likely a rate-limiting factor that drives a compensatory response in gait initiation. Additional research is needed to explore the neural correlates of HD-related movement scaling.

scholarly journals Cognitive and Motor Norms for Huntington’s Disease

2020 ◽  
Vol 35 (6) ◽  
pp. 671-682
Author(s):  
James A Mills ◽  
Jeffrey D Long ◽  
Amrita Mohan ◽  
Jennifer J Ware ◽  
Cristina Sampaio
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scale ◽  
Word Reading ◽  
Natural Aging ◽  
Color Naming ◽  
Stroop Interference ◽  
Healthy Controls ◽  
Aging Effects ◽  
Motor Score

Abstract Background The progression of Huntington’s disease (HD) for gene-expanded carriers is well-studied. Natural aging effects, however, are not often considered in the evaluation of HD progression. Objective To examine the effects of natural aging for healthy controls and to develop normative curves by age, sex, and education from the distribution of observed scores for the Symbol Digit Modalities Test, Stroop Word Reading Test, Stroop Color Naming Test, Stroop Interference Test, Total Motor Score, and Total Functional Capacity (TFC) from the Unified Huntington’s Disease Rating Scale (UHDRS) along with a composite score. Methods After combining longitudinal REGISTRY and Enroll-HD data, we used quantile regression and natural cubic splines for age to fit models for healthy controls (N = 3,394; N observations = 8,619). Normative curves were estimated for the 0.05, 0.25, 0.50, 0.75, and 0.95 quantiles. Two types of reference curves were considered: unconditional curves were dependent on age alone, whereas conditional curves were dependent on age and other covariates, namely sex and education. Results Conditioning on education was necessary for the Symbol Digit, Stroop Word, Stroop Color, Stroop Interference, and composite UHDRS. Unconditional curves were sufficient for the Total Motor Score. TFC was unique in that the curve was constant over age with its intercept at the maximum score (TFC = 13). For all measures, sex effects were minimal, so conditioning on sex was unwarranted. Conclusions Extreme quantile estimates for each measure can be considered as boundaries for natural aging and scores falling beyond these thresholds are likely the result of disease progression. Normative curves and tables are developed and can serve as references for clinical characterization in HD.

Reduced autonomic responsiveness to gambling task losses in Huntington's disease

2004 ◽  
Vol 10 (2) ◽  
pp. 239-245 ◽  
Author(s):  
MEGHAN C. CAMPBELL ◽  
JULIE C. STOUT ◽  
PETER R. FINN
Keyword(s):  
Decision Making ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Decision Task ◽  
Healthy Controls ◽  
Gambling Task ◽  
Risky Decision ◽  
Skin Conductance Responses ◽  
Decision Making Processes

We examined the possible role of autonomic activity in Huntington's disease (HD) during a risky decision making task. Skin conductance responses (SCRs) of 15 HD participants and 16 healthy controls were measured while they performed a computerized version of the Simulated Gambling Task (SGT). The results replicated our previous finding of a performance decrement in HD, and showed that HD was associated with an altered pattern of SCRs during the risky decision task. Specifically, the healthy controls produced increased SCRs following selections from the disadvantageous decks and following losing selections. In contrast, the SCRs of the HD group did not differentiate between wins and losses. These findings indicate a reduced impact of loss on decision-making processes under risky conditions in HD. (JINS, 2004, 10, 239–245.)

Assessing Impairment of Executive Function and Psychomotor Speed in Premanifest and Manifest Huntington’s Disease Gene-expansion Carriers

2015 ◽  
Vol 21 (3) ◽  
pp. 193-202 ◽  
Author(s):  
Ida Unmack Larsen ◽  
Tua Vinther-Jensen ◽  
Anders Gade ◽  
Jørgen Erik Nielsen ◽  
Asmus Vogel
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neuropsychological Testing ◽  
Distinct Group ◽  
Healthy Controls ◽  
Psychomotor Speed ◽  
Gene Expansion ◽  
Individual Scores ◽  
Hd Gene ◽  
Group Comparisons

AbstractExecutive functions (EF) and psychomotor speed (PMS) has been widely studied in Huntington’s disease (HD). Most studies have focused on finding markers of disease progression by comparing group means at different disease stages. Our aim was to investigate performances on nine measures of EF and PMS in a group of premanifest and manifest HD-gene expansion carriers and to investigate which measures were most sensitive for assessment of individual patients by analyzing frequencies of impaired performances relative to healthy controls. We recruited HD gene-expansion carriers, 48 manifest and 50 premanifest and as controls 39 healthy gene-expansion negative individuals. All participants underwent neurological examination and neuropsychological testing with nine cognitive measures. The frequency of impairment was investigated using cutoff scores. In group comparisons the manifest HD gene-expansion carriers scored significantly worse than controls on all tests and in classification of individual scores the majority of scores were classified as probably impaired (10th percentile) or impaired (5th percentile) with Symbol Digit Modalities Test (SDMT) being the most frequently impaired. Group comparisons of premanifest HD gene-expansion carriers and healthy controls showed significant differences on SDMT and Alternating fluency tests. Nevertheless the frequencies of probably impaired and impaired scores on individual tests were markedly higher for Alternating and Lexical fluency tests than for SDMT. We found distinct group differences in frequency of impairment on measures of EF and PMS in manifest and premanifest HD gene-expansion carriers. Our results indicate to what degree these measures can be expected to be clinically impaired. (JINS, 2015, 21, 1–10)

scholarly journals Altered Gut Microbiota Related to Inflammatory Responses in Patients With Huntington’s Disease

2021 ◽  
Vol 11 ◽  
Author(s):  
Gang Du ◽  
Wei Dong ◽  
Qing Yang ◽  
Xueying Yu ◽  
Jinghong Ma ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Clinical Characteristics ◽  
Inflammatory Responses ◽  
Plasma Concentrations ◽  
Fecal Microbiota ◽  
Healthy Controls ◽  
Fecal Microbiome ◽  
Cytokine Responses ◽  
Α Diversity

Emerging evidence indicates that gut dysbiosis may play a regulatory role in the onset and progression of Huntington’s disease (HD). However, any alterations in the fecal microbiome of HD patients and its relation to the host cytokine response remain unknown. The present study investigated alterations and host cytokine responses in patients with HD. We enrolled 33 HD patients and 33 sex- and age- matched healthy controls. Fecal microbiota communities were determined through 16S ribosomal DNA gene sequencing, from which we analyzed fecal microbial richness, evenness, structure, and differential abundance of individual taxa between HD patients and healthy controls. HD patients were evaluated for their clinical characteristics, and the relationships of fecal microbiota with these clinical characteristics were analyzed. Plasma concentrations of interferon gamma (IFN-γ), interleukin 1 beta (IL-1β), IL-2, IL-4, IL-6, IL-8, IL-10, IL-12p70, IL-13, and tumor necrosis factor alpha were measured by Meso Scale Discovery (MSD) assays, and relationships between microbiota and cytokine levels were analyzed in the HD group. HD patients showed increased α-diversity (richness), β-diversity (structure), and altered relative abundances of several taxa compared to those in healthy controls. HD-associated clinical characteristics correlated with the abundances of components of fecal microbiota at the genus level. Genus Intestinimonas was correlated with total functional capacity scores and IL-4 levels. Our present study also revealed that genus Bilophila were negatively correlated with proinflammatory IL-6 levels. Taken together, our present study represents the first to demonstrate alterations in fecal microbiota and inflammatory cytokine responses in HD patients. Further elucidation of interactions between microbial and host immune responses may help to better understand the pathogenesis of HD.

scholarly journals Anticipatory Postural Adjustments During Gait Initiation in Stroke Patients

2019 ◽  
Vol 10 ◽  
Author(s):  
Arnaud Delafontaine ◽  
Thomas Vialleron ◽  
Tarek Hussein ◽  
Eric Yiou ◽  
Jean-Louis Honeine ◽  
...  
Keyword(s):  
Anticipatory Postural Adjustments ◽  
Gait Initiation ◽  
Stroke Patients ◽  
Postural Adjustments
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