F15 Visual-cognitive impairment in asymptomatic and symptomatic carriers of huntington’s disease (HD)

2021 ◽  
Author(s):  
Miriam Turuelo ◽  
Rocio Del Pino ◽  
Maria Ángeles Acera ◽  
María Díez-Cirarda ◽  
Tamara Fernández ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Visual Cognitive

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 Revisiting the neuropsychiatry of Huntington's disease

2016 ◽  
Vol 10 (4) ◽  
pp. 261-266 ◽  
Author(s):  
Antonio Lucio Teixeira ◽  
Leonardo Cruz de Souza ◽  
Natalia Pessoa Rocha ◽  
Erin Furr-Stimming ◽  
Edward C. Lauterbach
Keyword(s):  
Cognitive Impairment ◽  
Neurodegenerative Disease ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Functional Capacity ◽  
Autosomal Dominant ◽  
Neuropsychiatric Symptoms ◽  
Executive Dysfunction ◽  
Behavioral Symptoms ◽  
Motor Symptoms

ABSTRACT Huntington's disease (HD) is an autosomal dominant neurodegenerative disease classified under the choreas. Besides motor symptoms, HD is marked by cognitive and behavioral symptoms, impacting patients' functional capacity. The progression of cognitive impairment and neuropsychiatric symptoms occur in parallel with neurodegeneration. The nature of these symptoms is very dynamic, and the major clinical challenges include executive dysfunction, apathy, depression and irritability. Herein, we provide a focused updated review on the cognitive and psychiatric features of HD.

scholarly journals Evaluation of Cognition and Cortical Excitability in Huntington’s Disease

2018 ◽  
Vol 45 (2) ◽  
pp. 176-181 ◽  
Author(s):  
Nitish Kamble ◽  
M. Netravathi ◽  
B.C. Nagaraju ◽  
Abhishek Lenka ◽  
Keshav Kumar ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cortical Excitability ◽  
Short Interval ◽  
Silent Period ◽  
Conduction Time ◽  
Resting Motor Threshold ◽  
Significant Difference ◽  
Significant Impairment

AbstractBackground: Recent advances in neurophysiological techniques have contributed to our understanding of the pathophysiology of Huntington’s disease (HD). Studies of the motor cortical excitability and central motor pathways have shown variable results. Objectives: Our aims were to evaluate the cortical excitability changes in HD using transcranial magnetic stimulation (TMS) and correlate the changes with cognitive impairment. Methods: The study included 32 HD patients and 30 age- and gender-matched controls. The demographic and clinical profiles of the patients were recorded. All subjects were evaluated by TMS and resting motor threshold (RMT), central motor conduction time (CMCT), silent period (SP), short-interval intracortical inhibition (SICI), and intracortical facilitation were determined. A battery of neuropsychological tests was administered to all subjects. Results: The mean age of the patients was 42.1±14.1 years, and that of controls 39.4±12.4 years (p=0.61). There was no significant difference in RMT and CMCT between the two groups. There was a mild prolongation of the contralateral SP in HD, but it was not significant. SICI was significantly reduced in HD (p<0.0001). A significant impairment in attention, verbal fluency, executive function, visuospatial function, learning, and memory was observed in HD patients. However, there was no correlation between cortical excitability changes and cognitive impairment. Conclusions: TMS is a valuable method of evaluating cortical excitability changes in HD. These patients have reduced SICI and significant impairment of cognition in multiple domains.

scholarly journals Deuterium‐reinforced linoleic acid lowers lipid peroxidation and mitigates cognitive impairment in the Q140 knock in mouse model of Huntington's disease

FEBS Journal ◽  
2018 ◽  
Vol 285 (16) ◽  
pp. 3002-3012 ◽  
Author(s):  
Asa Hatami ◽  
Chunni Zhu ◽  
Aroa Relaño‐Gines ◽  
Chris Elias ◽  
Arpine Galstyan ◽  
...  
Keyword(s):  
Lipid Peroxidation ◽  
Cognitive Impairment ◽  
Linoleic Acid ◽  
Mouse Model ◽  
Huntington's Disease ◽  
Huntington’S Disease

Selective cognitive impairment in the YAC128 Huntington's disease mouse

2012 ◽  
Vol 88 (2-3) ◽  
pp. 121-129 ◽  
Author(s):  
Simon P. Brooks ◽  
Nari Janghra ◽  
Gemma V. Higgs ◽  
Zubeyde Bayram-Weston ◽  
Andreas Heuer ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Huntington's Disease ◽  
Huntington’S Disease

scholarly journals Apathy predicts rate of cognitive decline over 24 months in premanifest Huntington's disease

2020 ◽  
pp. 1-7
Author(s):  
S. C. Andrews ◽  
D. R. Langbehn ◽  
D. Craufurd ◽  
A. Durr ◽  
B. R. Leavitt ◽  
...  
Keyword(s):  
Cognitive Impairment ◽  
Cognitive Decline ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Longitudinal Design ◽  
Motor Symptom ◽  
Control Group ◽  
Severity Rating ◽  
Healthy Control ◽  
Prospective Longitudinal

Abstract Background Cognitive impairment is a core feature of Huntington's disease (HD), however, the onset and rate of cognitive decline is highly variable. Apathy is the most common neuropsychiatric symptom of HD, and is associated with cognitive impairment. The aim of this study was to investigate apathy as a predictor of subsequent cognitive decline over 2 years in premanifest and early HD, using a prospective, longitudinal design. Methods A total of 118 premanifest HD gene carriers, 111 early HD and 118 healthy control participants from the multi-centre TRACK-HD study were included. Apathy symptoms were assessed at baseline using the apathy severity rating from the Short Problem Behaviours Assessment. A composite of 12 outcome measures from nine cognitive tasks was used to assess cognitive function at baseline and after 24 months. Results In the premanifest group, after controlling for age, depression and motor signs, more apathy symptoms predicted faster cognitive decline over 2 years. In contrast, in the early HD group, more motor signs, but not apathy, predicted faster subsequent cognitive decline. In the control group, only older age predicted cognitive decline. Conclusions Our findings indicate that in premanifest HD, apathy is a harbinger for cognitive decline. In contrast, after motor onset, in early diagnosed HD, motor symptom severity more strongly predicts the rate of cognitive decline.

Montreal cognitive assessment for evaluating cognitive impairment in Huntington’s disease: a systematic review

CNS Spectrums ◽  
2020 ◽  
pp. 1-19
Author(s):  
Elena Cecilia Rosca ◽  
Mihaela Simu
Keyword(s):  
Cognitive Impairment ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Assessment ◽  
Cognitive Status ◽  
Montreal Cognitive Assessment ◽  
Cutoff Score ◽  
Index Test ◽  
Cross Sectional ◽  
Cross Sectional Studies

Abstract Objective This study aims to systematically review evidence of the accuracy of the Montreal Cognitive Assessment (MoCA) for evaluating the presence of cognitive impairment in patients with Huntington’s disease (HD) and to outline the quality and quantity of research evidence available about the use of the MoCA in this population. Methods We conducted a systematic literature review, searching four databases from inception until April 2020. Results We identified 26 studies that met the inclusion criteria: two case–control studies comparing the MoCA to a battery of tests, three studies comparing MoCA to Mini-Mental State Examination, two studies estimating the prevalence of cognitive impairment in individuals with HD and 19 studies or clinical trials in which the MoCA was used as an instrument for the cognitive assessment of participants with HD. We found no cross-sectional studies in which participants received the index test (MoCA) and a reference standard diagnostic assessment composed of an extensive neuropsychological battery. The publication period ranged from 2010 to 2020. Conclusions In patients with HD, the MoCA provides information about disturbances in general cognitive function. Even if the MoCA demonstrated good sensitivity and specificity when used at the recommended threshold score of 26, further cross-sectional studies are required to examine the optimum cutoff score for detecting cognitive impairments in patients with HD. Moreover, more studies are necessary to determine whether the MoCA adequately assesses cognitive status in individuals with HD.

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