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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 single cases.Areal volume reductions in CBS patients at t0 and from t0 to t1 overlaid on coronal sections of the JuBrain cytoarchitectonic atlas: Colored areas illustrate a) atrophy of four or more SD in patient 1 and b) patient 5.
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pone-0110326-g003: Atrophy patterns in l-CBS and r-CBS single cases.Areal volume reductions in CBS patients at t0 and from t0 to t1 overlaid on coronal sections of the JuBrain cytoarchitectonic atlas: Colored areas illustrate a) atrophy of four or more SD in patient 1 and b) patient 5.

Mentions: The two cases differed in the pattern of neural atrophy, both at the initial point of examination and during disease progression. In patient 1 (l-CBS), atrophy was mainly characterized by bilaterally affected primary somatosensory areas, superior parietal, intraparietal and temporal regions, followed by extending atrophy in orbitofrontal areas in the course of the disease (Figure 3a). In contrast, patient 5 (r-CBS) revealed bilateral atrophy predominantly in the premotor, primary motor and somatosensory cortex, followed by atrophy extending bilaterally to the superior parietal cortex and a worsening of already affected motor areas (Figure 3b). All atrophic brain regions, including the amount of deviation from the control group (at t0) and the amount of atrophy during disease progression (t0-t1) are listed in Appendix S6.


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 single cases.Areal volume reductions in CBS patients at t0 and from t0 to t1 overlaid on coronal sections of the JuBrain cytoarchitectonic atlas: Colored areas illustrate a) atrophy of four or more SD in patient 1 and b) patient 5.
© Copyright Policy
Related In: Results  -  Collection

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

pone-0110326-g003: Atrophy patterns in l-CBS and r-CBS single cases.Areal volume reductions in CBS patients at t0 and from t0 to t1 overlaid on coronal sections of the JuBrain cytoarchitectonic atlas: Colored areas illustrate a) atrophy of four or more SD in patient 1 and b) patient 5.
Mentions: The two cases differed in the pattern of neural atrophy, both at the initial point of examination and during disease progression. In patient 1 (l-CBS), atrophy was mainly characterized by bilaterally affected primary somatosensory areas, superior parietal, intraparietal and temporal regions, followed by extending atrophy in orbitofrontal areas in the course of the disease (Figure 3a). In contrast, patient 5 (r-CBS) revealed bilateral atrophy predominantly in the premotor, primary motor and somatosensory cortex, followed by atrophy extending bilaterally to the superior parietal cortex and a worsening of already affected motor areas (Figure 3b). All atrophic brain regions, including the amount of deviation from the control group (at t0) and the amount of atrophy during disease progression (t0-t1) are listed in Appendix S6.

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