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 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.

Quantitative assessment of daytime motor activity provides a responsive measure of functional decline in patients with Huntington's disease

2001 ◽  
Vol 16 (3) ◽  
pp. 481-488 ◽  
Author(s):  
J.P.P. van Vugt ◽  
S. Siesling ◽  
K.K.E. Piet ◽  
A.H. Zwinderman ◽  
H.A.M. Middelkoop ◽  
...  
Keyword(s):  
Motor Activity ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Quantitative Assessment ◽  
Functional Decline

The relationship between impairment of voluntary movements and cognitive impairment in Huntington’s disease

2009 ◽  
Vol 256 (10) ◽  
pp. 1629-1633 ◽  
Author(s):  
Jiří Klempíř ◽  
Olga Klempířová ◽  
Jan Štochl ◽  
Nataša Špačková ◽  
Jan Roth
Keyword(s):  
Cognitive Impairment ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Voluntary Movements ◽  
The Relationship

Profiles of motor and cognitive impairment in the transgenic rat model of Huntington's disease

2012 ◽  
Vol 88 (2-3) ◽  
pp. 223-236 ◽  
Author(s):  
Steven A. Fielding ◽  
Simon P. Brooks ◽  
Alexander Klein ◽  
Zubeyde Bayram-Weston ◽  
Lesley Jones ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rat Model ◽  
Transgenic Rat ◽  
Transgenic Rat Model

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 Laser Evoked Potentials in Early and Presymptomatic Huntington’s Disease

2016 ◽  
Vol 2016 ◽  
pp. 1-8 ◽  
Author(s):  
Marina de Tommaso ◽  
Giovanni Franco ◽  
Katia Ricci ◽  
Anna Montemurno ◽  
Vittorio Sciruicchio
Keyword(s):  
Evoked Potentials ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Functional Decline ◽  
Disease Onset ◽  
Laser Evoked Potentials ◽  
Clinical Onset ◽  
Pain Pathways ◽  
Potential Biomarker ◽  
Pain Symptoms

Pain was rarely studied in Huntington’s disease (HD). We presently aimed to extend our previous study on pain pathways functions by laser evoked potentials (LEPs) to a larger cohort of early unmedicated HD patients and a small group of presymptomatic HD (PHD) subjects. Forty-two early HD patients, 10 PHD patients, and 64 controls were submitted to LEPs by right-hand stimulation. Two series of 30 laser stimuli were delivered, and artifact-free responses were averaged. The N1, N2, and P2 latencies were significantly increased and the N2P2 amplitude significantly reduced in HD patients compared to controls. In the HD group, the LEPs abnormalities correlated with functional decline. PHD subjects showed a slight and insignificant increase in LEPs latencies, which was inversely correlated with the possible age of HD clinical onset. Data of the present study seem to suggest that the functional state of nociceptive pathways as assessed by LEPs may be a potential biomarker of disease onset and progression. The assessment of pain symptoms in premanifest and manifest HD may also open a new scenario in terms of subtle disturbances of pain processing, which may have a role in the global burden of the disease.

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