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Posterior cortical atrophy: Primary occipital variant.
Dror Shir1, Jonathan Graff-Radford1, Mary M Machulda2
1Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
European Journal of Neurology
|March 17, 2022
Summary
Posterior cortical atrophy (PCA) has a newly identified primary occipital variant. This variant presents with older age, color vision deficits, and distinct neuroimaging findings compared to other PCA subtypes.
Area of Science:
- Neurology
- Neuroscience
- Medical Imaging
Background:
- Posterior cortical atrophy (PCA) is an atypical Alzheimer's disease variant.
- PCA is characterized by visuospatial and visuoperceptual deficits.
- Established dorsal and ventral subtypes exist, with a potential primary occipital (caudal) variant suggested.
Purpose of the Study:
- To define the demographics, clinical features, and biomarker profile of the primary occipital variant of PCA.
- To differentiate the primary occipital PCA from other PCA subtypes.
Main Methods:
- Investigated 52 PCA patients using neuropsychological assessment, MRI, and PET scans (FDG-PET, amyloid-PET, tau-PET).
- Classified patients into "primary occipital" and "other PCA" subgroups based on FDG-PET criteria.
- Analyzed regional FDG-PET z-scores, global amyloid-PET, temporo-parietal FDG-PET, and temporal tau-PET.
Main Results:
- Identified 9 patients with primary occipital PCA; they were older and had more education.
- Primary occipital PCA patients showed worse color perception (Ishihara test) but less severe ideomotor apraxia (WAB praxis scale).
- Primary occipital PCA had higher FDG-PET meta-ROI but no differences in amyloid- or tau-PET compared to other PCA.
Conclusions:
- Primary occipital PCA is characterized by older age at onset and impaired color perception.
- This variant exhibits less severe ideomotor apraxia and reduced temporo-parietal hypometabolism.
- Findings differentiate primary occipital PCA from established PCA phenotypes.
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