Echogenicity of basal ganglia structures in different phenotypes of Huntington's disease

2014 ◽  
Vol 45 (01) ◽  
Author(s):  
C Krogias ◽  
R Hoffmann ◽  
K Straßburger-Krogias ◽  
P Klotz ◽  
G Ellrichmann ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Basal Ganglia Structures

Task-Set Switching Deficits in Early-Stage Huntington's Disease: Implications for Basal Ganglia Function

2003 ◽  
Vol 15 (5) ◽  
pp. 629-642 ◽  
Author(s):  
Adam R. Aron ◽  
Laura Watkins ◽  
Barbara J. Sahakian ◽  
Stephen Monsell ◽  
Roger A. Barker ◽  
...  
Keyword(s):  
Executive Function ◽  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Frontal Lobe ◽  
Task Switching ◽  
Early Stage ◽  
Button Press ◽  
Task Set ◽  
Basal Ganglia Structures

Executive functions are likely mediated by interconnected circuits including frontal lobe and basal ganglia structures. We assessed the executive function of task switching in patients with early-stage Huntington's disease (HD), a neurodegenerative disease affecting the basal ganglia. In two experiments, the HD patients had greater difficulty when switching than when repeating a task than matched controls, and this was true even when scaling for the overall slowing of the patients. In the first experiment, HD patients had a switching deficit even in a “pure” condition where they had to switch, predictably, and with substantial preparation time, between stimuli having only one possible response, indicating a switching deficit different from that for patients with Parkinson's disease or frontal lobe trauma, and possibly relating to inadequate activation of stimulus-response links or “response set.” In the more elaborate second experiment, we could not account for the switching deficit of the patients in terms of inadequate preparation in advance of a switch, deficient suppression of taskset processing from the preswitch trial, or impaired suppression of interference due to the presence of a competing task set. Instead, we found that part of the switching deficit was due to elevated reaction time and errors on switch trials for a repeated response (same button press as on preswitch trial) relative to an alternated response (different button press from preswitch trial). We argue that this elevated “repetition effect” for the HD patients is due to excessive inhibition of the justperformed response in advance of a switch. Alterations in the “response-setting” process alone (Experiment 1) and both the response-setting and “response inhibition” process (Experiment 2) probably arise from striatal pathology in HD, thus accounting for the task-switching deficits and showing how basal ganglia implemented response processes may underpin executive function.

E27 Different Huntington's Disease Phenotypes And Echogenicity Of Basal Ganglia Structures

2014 ◽  
Vol 85 (Suppl 1) ◽  
pp. A46-A46
Author(s):  
C. Saft ◽  
R. Hoffmann ◽  
K. Strassburger-Krogias ◽  
T. Lucke ◽  
S. Meves ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Phenotypes ◽  
Basal Ganglia Structures

Echogenicity of basal ganglia structures in different Huntington’s disease phenotypes

2014 ◽  
Vol 122 (6) ◽  
pp. 825-833 ◽  
Author(s):  
Carsten Saft ◽  
Rainer Hoffmann ◽  
Katrin Strassburger-Krogias ◽  
Thomas Lücke ◽  
Saskia H. Meves ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Phenotypes ◽  
Basal Ganglia Structures

scholarly journals Basal ganglia-cortical structural connectivity in Huntington's disease

2015 ◽  
Vol 36 (5) ◽  
pp. 1728-1740 ◽  
Author(s):  
Marianne J.U. Novak ◽  
Kiran K. Seunarine ◽  
Clare R. Gibbard ◽  
Peter McColgan ◽  
Bogdan Draganski ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Structural Connectivity

Basal Ganglia Circuit integrity Assessed in Working Memory in Huntington's Disease

NeuroImage ◽  
2009 ◽  
Vol 47 ◽  
pp. S115
Author(s):  
R.K. Pierson ◽  
L.J. Beglinger ◽  
V.A. Magnotta ◽  
J. Vaidya ◽  
H. Johnson ◽  
...  
Keyword(s):  
Working Memory ◽  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease

Cholecystokinin Content in the Basal Ganglia in Huntington's Disease

1985 ◽  
Vol 448 (1) ◽  
pp. 488-494 ◽  
Author(s):  
P. C. EMSON ◽  
D. DAWBARN ◽  
M. N. ROSSOR ◽  
J. F. REHFELD ◽  
P. BRUNDIN ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease

scholarly journals Abnormal eye movements in three types of chorea

2016 ◽  
Vol 74 (9) ◽  
pp. 761-766 ◽  
Author(s):  
Tiago Attoni ◽  
Rogério Beato ◽  
Serge Pinto ◽  
Francisco Cardoso
Keyword(s):  
Eye Movements ◽  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Continuous Flow ◽  
Muscle Contractions ◽  
Eye Motion ◽  
Sydenham's Chorea ◽  
Ocular Changes ◽  
Degenerative Processes

ABSTRACT Chorea is an abnormal movement characterized by a continuous flow of random muscle contractions. This phenomenon has several causes, such as infectious and degenerative processes. Chorea results from basal ganglia dysfunction. As the control of the eye movements is related to the basal ganglia, it is expected, therefore, that is altered in diseases related to chorea. Sydenham’s chorea, Huntington’s disease and neuroacanthocytosis are described in this review as basal ganglia illnesses that can present with abnormal eye movements. Ocular changes resulting from dysfunction of the basal ganglia are apparent in saccade tasks, slow pursuit, setting a target and anti-saccade tasks. The purpose of this article is to review the main characteristics of eye motion in these three forms of chorea.

Cognitive and Affective Empathy in Huntington’s Disease

2021 ◽  
pp. 1-12
Author(s):  
Arnau Puig-Davi ◽  
Saul Martinez-Horta ◽  
Frederic Sampedro ◽  
Andrea Horta-Barba ◽  
Jesus Perez-Perez ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Deficits ◽  
Perspective Taking ◽  
Neural Correlates ◽  
Affective Empathy ◽  
Grey Matter Volume ◽  
Empathy Deficits ◽  
Almost All

Background: Empathy is a multidimensional construct and a key component of social cognition. In Huntington’s disease (HD), little is known regarding the phenomenology and the neural correlates of cognitive and affective empathy, and regarding how empathic deficits interact with other behavioral and cognitive manifestations. Objective: To explore the cognitive and affective empathy disturbances and related behavioral and neural correlates in HD. Methods: Clinical and sociodemographic data were obtained from 36 healthy controls (HC) and 54 gene-mutation carriers (17 premanifest and 37 early-manifest HD). The Test of Cognitive and Affective Empathy (TECA) was used to characterize cognitive (CE) and affective empathy (AE), and to explore their associations with grey matter volume (GMV) and cortical thickness (Cth). Results: Compared to HC, premanifest participants performed significantly worse in perspective taking (CE) and empathic distress (AE). In symptomatic participants, scores were significantly lower in almost all the TECA subscales. Several empathy subscales were associated with the severity of apathy, irritability, and cognitive deficits. CE was associated with GMV in thalamic, temporal, and occipital regions, and with Cth in parietal and temporal areas. AE was associated with GMV in the basal ganglia, limbic, occipital, and medial orbitofrontal regions, and with Cth in parieto-occipital areas. Conclusion: Cognitive and affective empathy deficits are detectable early, are more severe in symptomatic participants, and involve the disruption of several fronto-temporal, parieto-occipital, basal ganglia, and limbic regions. These deficits are associated with disease severity and contribute to several behavioral symptoms, facilitating the presentation of maladaptive patterns of social interaction.

Changes in the brain

2020 ◽  
pp. 92-102
Author(s):  
Oliver Quarrell
Keyword(s):  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Corpus Striatum ◽  
Physical Features ◽  
The Brain

This chapter explains the change which can be seen in the structure of the brain of someone with Huntington’s disease (HD). There is a recognized pattern of damage which occurs. Several areas of the brain are affected but the brunt of the damage occurs in the basal ganglia especially the caudate and putamen nuclei, these are sometimes called the corpus striatum. There are complex connections between the areas of the brain: in the case of HD the direct and indirect pathways are important for understanding some of the physical features and why drugs which block the dopamine 2 receptors are used to treat chorea.

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