Cognitive Manifestations of Huntington Disease in Relation to Genetic Structure and Clinical Onset

Abstract

Cognitive decline appears to start before clinical onset of HD and is correlated with the number of trinucleotide repeats. Subsequent cognitive decline is primarily a function of number of years affected, although there is evidence that the presence of more trinucleotide repeats is associated with faster deterioration.

Executive dysfunction in early stages of Huntington's disease is associated with striatal and insular atrophy: A neuropsychological and voxel-based morphometric study

“…There is some evidence from previous studies that executive dysfunction in HD is possibly caused by disruption of the CBGTCC at the level of the striatum [28]. The present study confirmed in its neuropsychological test part that HD patients in early stages had executive dysfunction, in accordance with previous studies [14,15]; furthermore, this reduced capability was significantly correlated with a reduction of gray matter in the caudate nucleus as demonstrated in the ROI-based analysis. Subgroup analyses strengthened this finding by demonstrating extended signal changes covering the whole anatomical structure of the striatum in HD patients with marked executive dysfunction, whereas HD patients which were least affected in the neuropsychological tests showed only slight signal alterations with less robust findings in striatal regions.…”

Executive dysfunction in early stages of Huntington's disease is associated with striatal and insular atrophy: A neuropsychological and voxel-based morphometric study

“…There is some evidence from previous studies that executive dysfunction in HD is possibly caused by disruption of the CBGTCC at the level of the striatum [28]. The present study confirmed in its neuropsychological test part that HD patients in early stages had executive dysfunction, in accordance with previous studies [14,15]; furthermore, this reduced capability was significantly correlated with a reduction of gray matter in the caudate nucleus as demonstrated in the ROI-based analysis. Subgroup analyses strengthened this finding by demonstrating extended signal changes covering the whole anatomical structure of the striatum in HD patients with marked executive dysfunction, whereas HD patients which were least affected in the neuropsychological tests showed only slight signal alterations with less robust findings in striatal regions.…”

Non-Verbal and Verbal Fluency in Prodromal Huntington's Disease

“…Interestingly, all fluency abilities correlated significantly with PYTO. The significant relationships align with the results of Jason et al [ 66 ] who found overall cognitive functioning (a composite of different tasks) to be correlated with the number of CAG repeats. However, the composite measure of executive functioning (composite of Thurstone Word Fluency, Design Fluency, Wisconsin Card Sorting Test, Stroop Color, and Word Test) of Jason et al [ 66 ] was not significantly correlated with CAG repeats.…”

“…The significant relationships align with the results of Jason et al [ 66 ] who found overall cognitive functioning (a composite of different tasks) to be correlated with the number of CAG repeats. However, the composite measure of executive functioning (composite of Thurstone Word Fluency, Design Fluency, Wisconsin Card Sorting Test, Stroop Color, and Word Test) of Jason et al [ 66 ] was not significantly correlated with CAG repeats. This could be due to the differing degrees of cognitive demand in the elements of the composite of different executive functioning tasks or due to the fact that they did not include age in the analyses.…”

Use of the frontal assessment battery in evaluating executive dysfunction in patients with Huntington’s disease

“…The cognitive decline in patients with HD appears to start before the clinical onset of the disease and is characterized by memory loss and executive dysfunction [12,15,32,34]. However, it is not clear whether the FAB would be a good tool to differentiate patients in the initial stage of the disease from controls in our population.…”

Executive dysfunction in early stages of Huntington's disease is associated with striatal and insular atrophy: A neuropsychological and voxel-based morphometric study

“…There is some evidence from previous studies that executive dysfunction in HD is possibly caused by disruption of the CBGTCC at the level of the striatum [28]. The present study confirmed in its neuropsychological test part that HD patients in early stages had executive dysfunction, in accordance with previous studies [14,15]; furthermore, this reduced capability was significantly correlated with a reduction of gray matter in the caudate nucleus as demonstrated in the ROI-based analysis. Subgroup analyses strengthened this finding by demonstrating extended signal changes covering the whole anatomical structure of the striatum in HD patients with marked executive dysfunction, whereas HD patients which were least affected in the neuropsychological tests showed only slight signal alterations with less robust findings in striatal regions.…”

Non-Verbal and Verbal Fluency in Prodromal Huntington's Disease

“…Interestingly, all fluency abilities correlated significantly with PYTO. The significant relationships align with the results of Jason et al [ 66 ] who found overall cognitive functioning (a composite of different tasks) to be correlated with the number of CAG repeats. However, the composite measure of executive functioning (composite of Thurstone Word Fluency, Design Fluency, Wisconsin Card Sorting Test, Stroop Color, and Word Test) of Jason et al [ 66 ] was not significantly correlated with CAG repeats.…”

“…The significant relationships align with the results of Jason et al [ 66 ] who found overall cognitive functioning (a composite of different tasks) to be correlated with the number of CAG repeats. However, the composite measure of executive functioning (composite of Thurstone Word Fluency, Design Fluency, Wisconsin Card Sorting Test, Stroop Color, and Word Test) of Jason et al [ 66 ] was not significantly correlated with CAG repeats. This could be due to the differing degrees of cognitive demand in the elements of the composite of different executive functioning tasks or due to the fact that they did not include age in the analyses.…”

Use of the frontal assessment battery in evaluating executive dysfunction in patients with Huntington’s disease

“…The cognitive decline in patients with HD appears to start before the clinical onset of the disease and is characterized by memory loss and executive dysfunction [12,15,32,34]. However, it is not clear whether the FAB would be a good tool to differentiate patients in the initial stage of the disease from controls in our population.…”

Executive dysfunction in early stages of Huntington's disease is associated with striatal and insular atrophy: A neuropsychological and voxel-based morphometric study

“…There is some evidence from previous studies that executive dysfunction in HD is possibly caused by disruption of the CBGTCC at the level of the striatum [28]. The present study confirmed in its neuropsychological test part that HD patients in early stages had executive dysfunction, in accordance with previous studies [14,15]; furthermore, this reduced capability was significantly correlated with a reduction of gray matter in the caudate nucleus as demonstrated in the ROI-based analysis. Subgroup analyses strengthened this finding by demonstrating extended signal changes covering the whole anatomical structure of the striatum in HD patients with marked executive dysfunction, whereas HD patients which were least affected in the neuropsychological tests showed only slight signal alterations with less robust findings in striatal regions.…”

Non-Verbal and Verbal Fluency in Prodromal Huntington's Disease

“…Interestingly, all fluency abilities correlated significantly with PYTO. The significant relationships align with the results of Jason et al [ 66 ] who found overall cognitive functioning (a composite of different tasks) to be correlated with the number of CAG repeats. However, the composite measure of executive functioning (composite of Thurstone Word Fluency, Design Fluency, Wisconsin Card Sorting Test, Stroop Color, and Word Test) of Jason et al [ 66 ] was not significantly correlated with CAG repeats.…”

“…The significant relationships align with the results of Jason et al [ 66 ] who found overall cognitive functioning (a composite of different tasks) to be correlated with the number of CAG repeats. However, the composite measure of executive functioning (composite of Thurstone Word Fluency, Design Fluency, Wisconsin Card Sorting Test, Stroop Color, and Word Test) of Jason et al [ 66 ] was not significantly correlated with CAG repeats. This could be due to the differing degrees of cognitive demand in the elements of the composite of different executive functioning tasks or due to the fact that they did not include age in the analyses.…”

Use of the frontal assessment battery in evaluating executive dysfunction in patients with Huntington’s disease

“…The cognitive decline in patients with HD appears to start before the clinical onset of the disease and is characterized by memory loss and executive dysfunction [12,15,32,34]. However, it is not clear whether the FAB would be a good tool to differentiate patients in the initial stage of the disease from controls in our population.…”