Background: Huntington’s disease (HD) is characterized by a progressive multisystem neuronal atrophy in the brain. Apart from motor signs, cognitive symptoms, particularly executive dysfunctions, are proposed to be r...Background: Huntington’s disease (HD) is characterized by a progressive multisystem neuronal atrophy in the brain. Apart from motor signs, cognitive symptoms, particularly executive dysfunctions, are proposed to be recognizable in early stages of disease. The aim of the present study was to clarify if cognitive dysf unction in early stages of HD is correlated with loco-regional structural chang es in 3D-MRI. Methods: Twenty-five patients with genetically confirmed HD in e arly clinical stages were included in the study and underwent neuropsychological testing, i.e., the executive tasks Tower of Hanoi (ToH), Stroop Colour Word Int erference Test (STROOP), and modified Wisconsin Card Sorting Test (mWCST). High -resolution volume-rendering MRI scans (MP-RAGE) were acquired on a 1.5 T sca nner in all patients and were analyzed by statistical parametric mapping and vox el-based morphometry (VBM) in comparison to an age-matched control group. Resu lts: Group analysis of HD patients demonstrated robust regional decreases of gra y matter volumes (p < 0.05, corrected for multiple comparisons) in the caudate a nd the putamen bilaterally with a global maximum at Talairach coordinates 11/4/1 1 (Z-score = 7.06). Executive dysfunction was significantly correlated with the areas of highest significant differences out of VBM results which were located bilaterally in the caudate (ToH:r= 0.647, p < 0.001; STROOP:r= 0.503, p < 0.01; mWCST:r= 0.452, p < 0.05). Moreover, subgroup analyses revealed marked insular a trophy (Talairach coordinates 43/-3/1; Z-score = 5.64) in HD patients who perf ormed worse in the single executive tasks. Conclusion: Two aspects were most rem arkable in this correlational study: (i) striatal atrophy in HD patients in earl y stages plays an important role not only in impaired motor control but also in executive dysfunction, and (ii) extrastriatal cortical areas, i.e., the insular lobe, seem to be involved in executive dysfunction as assessed by neuropsycholog ical tests requiring for planning and problem solving, stimulus response selecti vity and concept formation.展开更多
文摘Background: Huntington’s disease (HD) is characterized by a progressive multisystem neuronal atrophy in the brain. Apart from motor signs, cognitive symptoms, particularly executive dysfunctions, are proposed to be recognizable in early stages of disease. The aim of the present study was to clarify if cognitive dysf unction in early stages of HD is correlated with loco-regional structural chang es in 3D-MRI. Methods: Twenty-five patients with genetically confirmed HD in e arly clinical stages were included in the study and underwent neuropsychological testing, i.e., the executive tasks Tower of Hanoi (ToH), Stroop Colour Word Int erference Test (STROOP), and modified Wisconsin Card Sorting Test (mWCST). High -resolution volume-rendering MRI scans (MP-RAGE) were acquired on a 1.5 T sca nner in all patients and were analyzed by statistical parametric mapping and vox el-based morphometry (VBM) in comparison to an age-matched control group. Resu lts: Group analysis of HD patients demonstrated robust regional decreases of gra y matter volumes (p < 0.05, corrected for multiple comparisons) in the caudate a nd the putamen bilaterally with a global maximum at Talairach coordinates 11/4/1 1 (Z-score = 7.06). Executive dysfunction was significantly correlated with the areas of highest significant differences out of VBM results which were located bilaterally in the caudate (ToH:r= 0.647, p < 0.001; STROOP:r= 0.503, p < 0.01; mWCST:r= 0.452, p < 0.05). Moreover, subgroup analyses revealed marked insular a trophy (Talairach coordinates 43/-3/1; Z-score = 5.64) in HD patients who perf ormed worse in the single executive tasks. Conclusion: Two aspects were most rem arkable in this correlational study: (i) striatal atrophy in HD patients in earl y stages plays an important role not only in impaired motor control but also in executive dysfunction, and (ii) extrastriatal cortical areas, i.e., the insular lobe, seem to be involved in executive dysfunction as assessed by neuropsycholog ical tests requiring for planning and problem solving, stimulus response selecti vity and concept formation.
