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Neuropsychological and brain volume differences in patients with left- and right-beginning corticobasal syndrome.

Jütten K, Pieperhoff P, Südmeyer M, Schleicher A, Ferrea S, Caspers S, Zilles K, Schnitzler A, Amunts K, Lux S - PLoS ONE (2014)

Bottom Line: A similar pattern of morphological and neuropsychological differences was found for the individual disease progression in l-CBS and r-CBS single cases.For similar durations of disease, volumetric grey matter loss related to CBS pathology appeared earlier and progressed faster in l-CBS than in r-CBS.Cognitive impairment in r-CBS was characterized by apraxia, and additional memory and perceptional deficits for l-CBS.

View Article: PubMed Central - PubMed

Affiliation: Institute of Neuroscience and Medicine (INM-1), Research Centre Jülich, Jülich, Germany.

ABSTRACT

Background: Corticobasal Syndrome (CBS) is a rare neurodegenerative syndrome characterized by unilaterally beginning frontoparietal and basal ganglia atrophy. The study aimed to prove the hypothesis that there are differences in hemispheric susceptibility to disease-related changes.

Methods: Two groups of CBS patients with symptoms starting either on the left or right body side were investigated. Groups consisted of four patients each and were matched for sex, age and disease duration. Patient groups and a group of eight healthy age-matched controls were analyzed using deformation field morphometry and neuropsychological testing. To further characterize individual disease progression regarding brain atrophy and neuropsychological performance, two female, disease duration-matched patients differing in initially impaired body side were followed over six months.

Results: A distinct pattern of neural atrophy and neuropsychological performance was revealed for both CBS: Patients with initial right-sided impairment (r-CBS) revealed atrophy predominantly in frontoparietal areas and showed, except from apraxia, no other cognitive deficits. In contrast, patients with impairment of the left body side (l-CBS) revealed more widespread atrophy, extending from frontoparietal to orbitofrontal and temporal regions; and apraxia, perceptional and memory deficits could be found. A similar pattern of morphological and neuropsychological differences was found for the individual disease progression in l-CBS and r-CBS single cases.

Conclusions: For similar durations of disease, volumetric grey matter loss related to CBS pathology appeared earlier and progressed faster in l-CBS than in r-CBS. Cognitive impairment in r-CBS was characterized by apraxia, and additional memory and perceptional deficits for l-CBS.

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Related in: MedlinePlus

Atrophy patterns in l-CBS and r-CBS groups compared to controls.a) Volumetric differences between patients and controls overlaid on coronal sections of the JuBrain cytoarchitectonic atlas. Colored areas illustrate significant volume reductions in l-CBS (blue), r-CBS (green) or in both CBS groups (pink). b) Each patient's volume reduction (in SD) compared to the control group. Patients 1 to 8 are listed from left to right according to Table 1, with l-CBS patients in blue and r-CBS patients in green. For brain areas that were found to be atrophic in group statistics, titles of the diagrams are printed in blue for the l-CBS group, in green for the r-CBS group and pink for overlap between the l-CBS and r-CBS group. Significant volume reductions of 2.58 SD or more are shaded in grey.
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pone-0110326-g002: Atrophy patterns in l-CBS and r-CBS groups compared to controls.a) Volumetric differences between patients and controls overlaid on coronal sections of the JuBrain cytoarchitectonic atlas. Colored areas illustrate significant volume reductions in l-CBS (blue), r-CBS (green) or in both CBS groups (pink). b) Each patient's volume reduction (in SD) compared to the control group. Patients 1 to 8 are listed from left to right according to Table 1, with l-CBS patients in blue and r-CBS patients in green. For brain areas that were found to be atrophic in group statistics, titles of the diagrams are printed in blue for the l-CBS group, in green for the r-CBS group and pink for overlap between the l-CBS and r-CBS group. Significant volume reductions of 2.58 SD or more are shaded in grey.

Mentions: Further exploration of the amount of local volume differences between l-CBS and r-CBS groups revealed correspondences and differences in the pattern of atrophy (Figure 2a). Primary motor areas were affected in both groups in the respective hemisphere contralateral to the impaired limb. However, atrophy in the l-CBS group affected more brain regions and extended partly bilaterally to primary motor and orbitofrontal regions. Moreover, primary and secondary somatosensory areas of the contralateral hemisphere were affected in l-CBS only, in addition to the intraparietal sulcus. In contrast to l-CBS, superior parietal areas of the ipsilateral hemisphere showed more atrophy in the r-CBS group (Appendix S5). Each patient's contribution to brain morphological group differences is visualized in Figure 2b.


Neuropsychological and brain volume differences in patients with left- and right-beginning corticobasal syndrome.

Jütten K, Pieperhoff P, Südmeyer M, Schleicher A, Ferrea S, Caspers S, Zilles K, Schnitzler A, Amunts K, Lux S - PLoS ONE (2014)

Atrophy patterns in l-CBS and r-CBS groups compared to controls.a) Volumetric differences between patients and controls overlaid on coronal sections of the JuBrain cytoarchitectonic atlas. Colored areas illustrate significant volume reductions in l-CBS (blue), r-CBS (green) or in both CBS groups (pink). b) Each patient's volume reduction (in SD) compared to the control group. Patients 1 to 8 are listed from left to right according to Table 1, with l-CBS patients in blue and r-CBS patients in green. For brain areas that were found to be atrophic in group statistics, titles of the diagrams are printed in blue for the l-CBS group, in green for the r-CBS group and pink for overlap between the l-CBS and r-CBS group. Significant volume reductions of 2.58 SD or more are shaded in grey.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4214821&req=5

pone-0110326-g002: Atrophy patterns in l-CBS and r-CBS groups compared to controls.a) Volumetric differences between patients and controls overlaid on coronal sections of the JuBrain cytoarchitectonic atlas. Colored areas illustrate significant volume reductions in l-CBS (blue), r-CBS (green) or in both CBS groups (pink). b) Each patient's volume reduction (in SD) compared to the control group. Patients 1 to 8 are listed from left to right according to Table 1, with l-CBS patients in blue and r-CBS patients in green. For brain areas that were found to be atrophic in group statistics, titles of the diagrams are printed in blue for the l-CBS group, in green for the r-CBS group and pink for overlap between the l-CBS and r-CBS group. Significant volume reductions of 2.58 SD or more are shaded in grey.
Mentions: Further exploration of the amount of local volume differences between l-CBS and r-CBS groups revealed correspondences and differences in the pattern of atrophy (Figure 2a). Primary motor areas were affected in both groups in the respective hemisphere contralateral to the impaired limb. However, atrophy in the l-CBS group affected more brain regions and extended partly bilaterally to primary motor and orbitofrontal regions. Moreover, primary and secondary somatosensory areas of the contralateral hemisphere were affected in l-CBS only, in addition to the intraparietal sulcus. In contrast to l-CBS, superior parietal areas of the ipsilateral hemisphere showed more atrophy in the r-CBS group (Appendix S5). Each patient's contribution to brain morphological group differences is visualized in Figure 2b.

Bottom Line: A similar pattern of morphological and neuropsychological differences was found for the individual disease progression in l-CBS and r-CBS single cases.For similar durations of disease, volumetric grey matter loss related to CBS pathology appeared earlier and progressed faster in l-CBS than in r-CBS.Cognitive impairment in r-CBS was characterized by apraxia, and additional memory and perceptional deficits for l-CBS.

View Article: PubMed Central - PubMed

Affiliation: Institute of Neuroscience and Medicine (INM-1), Research Centre Jülich, Jülich, Germany.

ABSTRACT

Background: Corticobasal Syndrome (CBS) is a rare neurodegenerative syndrome characterized by unilaterally beginning frontoparietal and basal ganglia atrophy. The study aimed to prove the hypothesis that there are differences in hemispheric susceptibility to disease-related changes.

Methods: Two groups of CBS patients with symptoms starting either on the left or right body side were investigated. Groups consisted of four patients each and were matched for sex, age and disease duration. Patient groups and a group of eight healthy age-matched controls were analyzed using deformation field morphometry and neuropsychological testing. To further characterize individual disease progression regarding brain atrophy and neuropsychological performance, two female, disease duration-matched patients differing in initially impaired body side were followed over six months.

Results: A distinct pattern of neural atrophy and neuropsychological performance was revealed for both CBS: Patients with initial right-sided impairment (r-CBS) revealed atrophy predominantly in frontoparietal areas and showed, except from apraxia, no other cognitive deficits. In contrast, patients with impairment of the left body side (l-CBS) revealed more widespread atrophy, extending from frontoparietal to orbitofrontal and temporal regions; and apraxia, perceptional and memory deficits could be found. A similar pattern of morphological and neuropsychological differences was found for the individual disease progression in l-CBS and r-CBS single cases.

Conclusions: For similar durations of disease, volumetric grey matter loss related to CBS pathology appeared earlier and progressed faster in l-CBS than in r-CBS. Cognitive impairment in r-CBS was characterized by apraxia, and additional memory and perceptional deficits for l-CBS.

Show MeSH
Related in: MedlinePlus