TY - JOUR
T1 - Posterior cortical atrophy
T2 - Primary occipital variant
AU - Shir, Dror
AU - Graff-Radford, Jonathan
AU - Machulda, Mary M.
AU - Pham, Nha Trang Thu
AU - Jack, Clifford R.
AU - Lowe, Val J.
AU - Whitwell, Jennifer L.
AU - Josephs, Keith A.
N1 - Funding Information:
V.J.L. receives research support from GE Healthcare, Siemens Molecular Imaging, AVID Radiopharmaceuticals, and the National Institutes of Health (NIH) (National Institute on Aging [NIA], National Cancer Institute [NCI]) and consults for Bayer Schering, Piramal Life Sciences, Life Molecular Imaging, Eisai, Inc., AVID Radiopharmaceuticals, and Merck Research. The other authors declare no financial or other conflicts of interest. J.G.‐R. is funded by the NIH and serves on the editorial board for Neurology.
Publisher Copyright:
© 2022 European Academy of Neurology.
PY - 2022/7
Y1 - 2022/7
N2 - Background: Posterior cortical atrophy (PCA) is one of the atypical Alzheimer's disease variants, characterized by predominant visuospatial and visuoperceptual deficits, with established dorsal and ventral subtypes. A third primary occipital (caudal) variant has been suggested. We aimed to determine its demographics, clinical manifestations, and biomarker findings. Methods: Fifty-two PCA patients were investigated. Patients underwent neuropsychological assessment, magnetic resonance imaging, and fluorodeoxyglucose (FDG)-, amyloid-, and tau-positron emission tomography (tau-PET) scans. Normalized regional FDG-PET values were represented as z-scores relative to a control population. Patients were divided into “primary occipital” and “other PCA” subgroups according to FDG-PET-defined criteria, with primary occipital defined as patients in which the z-scores for occipital subregions were at least one standard deviation lower (SD) (i.e., more abnormal) than the z-scores in all other brain regions. Global amyloid-PET, temporo-parietal FDG-PET, and temporal tau-PET regions-of-interest (ROIs) were calculated. Results: Nine patients were classified as primary occipital; they were older (p = 0.034) and had more years of education (p = 0.007) than other PCA patients. The primary occipital group performed worse on the Ishihara test for color perception (p < 0.001), while other PCA patients performed worse on the Western Aphasia Battery (WAB) praxis scale (p = 0.005). Overall neuropsychiatric symptom burden was lower in the primary occipital group (p < 0.001). The FDG-PET meta-ROI was higher in the primary occipital subtype (p = 0.006), but no differences were observed in amyloid- and tau-PET. Conclusions: Our findings suggest that primary occipital PCA is characterized by an older age at onset, more color perception dysfunction, less severe ideomotor apraxia, and less hypometabolism in temporo-parietal meta-ROI compared to established phenotypes.
AB - Background: Posterior cortical atrophy (PCA) is one of the atypical Alzheimer's disease variants, characterized by predominant visuospatial and visuoperceptual deficits, with established dorsal and ventral subtypes. A third primary occipital (caudal) variant has been suggested. We aimed to determine its demographics, clinical manifestations, and biomarker findings. Methods: Fifty-two PCA patients were investigated. Patients underwent neuropsychological assessment, magnetic resonance imaging, and fluorodeoxyglucose (FDG)-, amyloid-, and tau-positron emission tomography (tau-PET) scans. Normalized regional FDG-PET values were represented as z-scores relative to a control population. Patients were divided into “primary occipital” and “other PCA” subgroups according to FDG-PET-defined criteria, with primary occipital defined as patients in which the z-scores for occipital subregions were at least one standard deviation lower (SD) (i.e., more abnormal) than the z-scores in all other brain regions. Global amyloid-PET, temporo-parietal FDG-PET, and temporal tau-PET regions-of-interest (ROIs) were calculated. Results: Nine patients were classified as primary occipital; they were older (p = 0.034) and had more years of education (p = 0.007) than other PCA patients. The primary occipital group performed worse on the Ishihara test for color perception (p < 0.001), while other PCA patients performed worse on the Western Aphasia Battery (WAB) praxis scale (p = 0.005). Overall neuropsychiatric symptom burden was lower in the primary occipital group (p < 0.001). The FDG-PET meta-ROI was higher in the primary occipital subtype (p = 0.006), but no differences were observed in amyloid- and tau-PET. Conclusions: Our findings suggest that primary occipital PCA is characterized by an older age at onset, more color perception dysfunction, less severe ideomotor apraxia, and less hypometabolism in temporo-parietal meta-ROI compared to established phenotypes.
KW - Alzheimer's disease
KW - posterior cortical atrophy
KW - visuospatial impairment
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U2 - 10.1111/ene.15327
DO - 10.1111/ene.15327
M3 - Article
C2 - 35298068
AN - SCOPUS:85127228094
VL - 29
SP - 2138
EP - 2143
JO - European Journal of Neurology
JF - European Journal of Neurology
SN - 1351-5101
IS - 7
ER -