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Functional neural substrates of posterior cortical atrophy patients

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Abstract

Posterior cortical atrophy (PCA) is a neurodegenerative syndrome in which the most pronounced pathologic involvement is in the occipito-parietal visual regions. Herein, we aimed to better define the cortical reflection of this unique syndrome using a thorough battery of behavioral and functional MRI (fMRI) tests. Eight PCA patients underwent extensive testing to map their visual deficits. Assessments included visual functions associated with lower and higher components of the cortical hierarchy, as well as dorsal- and ventral-related cortical functions. fMRI was performed on five patients to examine the neuronal substrate of their visual functions. The PCA patient cohort exhibited stereopsis, saccadic eye movements and higher dorsal stream-related functional impairments, including simultant perception, image orientation, figure-from-ground segregation, closure and spatial orientation. In accordance with the behavioral findings, fMRI revealed intact activation in the ventral visual regions of face and object perception while more dorsal aspects of perception, including motion and gestalt perception, revealed impaired patterns of activity. In most of the patients, there was a lack of activity in the word form area, which is known to be linked to reading disorders. Finally, there was evidence of reduced cortical representation of the peripheral visual field, corresponding to the behaviorally assessed peripheral visual deficit. The findings are discussed in the context of networks extending from parietal regions, which mediate navigationally related processing, visually guided actions, eye movement control and working memory, suggesting that damage to these networks might explain the wide range of deficits in PCA patients.

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Conflicts of interest

The authors report no financial disclosure.

Ethical standards

The study was approved by the Hadassah Hebrew University Medical Center Ethics Committee and all patients signed a consent form allowing their participation in the study. The research has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Authors and Affiliations

  1. fMRI unit, Department of Neurology, The Agnes Ginges Center for Human Neurogenetics, Hadassah Hebrew-University Medical Center, Hadassah Ein Karem, P.O.B 12000, 91120, Jerusalem, Israel

    H. Shames, N. Raz & Netta Levin

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  1. H. Shames
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  2. N. Raz
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  3. Netta Levin
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Correspondence to Netta Levin.

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Shames, H., Raz, N. & Levin, N. Functional neural substrates of posterior cortical atrophy patients. J Neurol 262, 1751–1761 (2015). https://doi.org/10.1007/s00415-015-7774-8

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