Abstract
BackgroundCorticobasal syndrome (CBS) is a multifaceted neurodegenerative disorder characterized by a combination of motor and cognitive deficits. Several different pathological entities, including Alzheimer’s pathology, have been described in association with CBS. The present study aimed to establish clinical, neuropsychological, and neuroimaging features that could be useful in the distinction of CBS due to AD pathology from other CBS cases in life based on [11C] Pittsburgh Compound B positron emission tomography (PiB-PET) status.MethodsPatients with CBS were prospectively recruited from a specialized cognitive disorders clinic. All patients underwent detailed clinical and neuropsychological assessment, with structural imaging using voxel-based analysis of magnetic resonance imaging. Alzheimer’s pathology was detected using PiB-PET imaging, and PiB-positive and PiB-negative groups were compared.ResultsFourteen CBS patients meeting defined criteria were included (7 male, 7 female; mean age 66.1+/−6.9 years; median symptom duration was 35.5+/−22.6 months) and compared to 20 matched control subjects. Of the 14 patients, 4 were PiB-positive and 10 PiB-negative. There were no significant differences between PiB-positive and PiB-negative CBS patients in age, gender, education, symptom duration, or motor features. PiB-positive patients had greater visuospatial deficits, a higher rate of sentence repetition impairment, and more functional decline. Voxel-based morphometry analyses demonstrated extensive peri-insular and post-central atrophy in both groups, but PiB-positive patients had atrophy that extended to include the posterior part of the left superior temporal gyrus.ConclusionsVisuospatial function, aspects of language, and the pattern of cerebral atrophy may be useful in distinguishing patients with CBS due to underlying AD pathology.
Highlights
[5] In addition, cognitive deficits with progressive disturbance of language and behavior are regarded as core features of the syndrome. [3,6,7,8] Pathologically, early studies reported mainly tau positive intra-neuronal inclusions, but Tar DNA binding protein-43 (TDP-43) positive inclusions and Alzheimer’s disease (AD) pathology have been increasingly recognized. [3,9,10,11,12] The ability to accurately detect underlying pathology early in the course of Corticobasal syndrome (CBS) will be crucial when effective therapies are developed
The positron emission tomography (PET) ligand [11C] Pittsburgh Compound B (PiB) was developed to detect fibrillar bamyloid peptide, which is a characteristic feature of AD pathology
The present study aimed to establish clinical, neuropsychological, and neuroimaging features that could be useful in the distinction of CBS due to AD pathology from other CBS cases in life based on PiB status
Summary
Corticobasal syndrome (CBS) is characterized by marked clinical and pathological heterogeneity. [1,2,3,4] While often considered an atypical parkinsonian syndrome, other motor system deficits are common and include asymmetrical limb apraxia – which may be severe and dominate the presentation, dystonia, and myoclonus. [5] In addition, cognitive deficits with progressive disturbance of language and behavior are regarded as core features of the syndrome. [3,6,7,8] Pathologically, early studies reported mainly tau positive intra-neuronal inclusions, but Tar DNA binding protein-43 (TDP-43) positive inclusions and Alzheimer’s disease (AD) pathology have been increasingly recognized. [3,9,10,11,12] The ability to accurately detect underlying pathology early in the course of CBS will be crucial when effective therapies are developed. The positron emission tomography (PET) ligand [11C] Pittsburgh Compound B (PiB) was developed to detect fibrillar bamyloid peptide, which is a characteristic feature of AD pathology. The present study aimed to establish clinical, neuropsychological, and neuroimaging features that could be useful in the distinction of CBS due to AD pathology from other CBS cases in life based on PiB status. Corticobasal syndrome (CBS) is a multifaceted neurodegenerative disorder characterized by a combination of motor and cognitive deficits. The present study aimed to establish clinical, neuropsychological, and neuroimaging features that could be useful in the distinction of CBS due to AD pathology from other CBS cases in life based on [11C] Pittsburgh Compound B positron emission tomography (PiB-PET) status
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
