Abstract
Objective: Posterior Cortical Atrophy (PCA) is a neurodegenerative disease characterized predominantly by visual impairment. However, diagnosis of PCA remains complicated with an interval of several years between initial reporting of symptoms and diagnosis. The aim of the present study is to define if patients' visual and gestural complaints are consistent with their clinical profile.Method: An evaluation of daily visual problems as well as a full neuropsychological assessment and FDG-PET were performed in 15 PCA patients. We compared glucose metabolism between these PCA patients and 18 healthy controls. Correlation analyses were conducted in PCA patients between visual and gestural complaint, clinical impairments, and brain glucose metabolism.Results: Major impairment of cognitive functions was detected in PCA patients specifically in visual domains. Positive correlations were found between visual impairments and hypometabolism in the right temporo-parieto-occipital cortices. However, no correlation was found between complaint and visual impairment in PCA patients.Discussion: Our main results suggest a consistent relationship between clinical impairment and brain metabolism. However, the patient's complaint and visual performance are not linked. Combining the literature and our results, it seems that patients are generally aware of difficulties but misinterpret them. This misinterpretation may be responsible for the delayed diagnosis.
Highlights
Posterior cortical atrophy (PCA) is characterized by an insidious onset, gradual progression and prominent early disturbance of visual functions [1]
We explored the link between complaint and brain glucose metabolism since literature suggests that a decrease in brain glucose metabolism explains cognitive impairment in PCA
PCA patients and HC were comparable in age (PCA: 66 [10], HC: 68 [6], p = 0.38), level of education in years
Summary
Posterior cortical atrophy (PCA) is characterized by an insidious onset, gradual progression and prominent early disturbance of visual functions [1]. PCA diagnosis remains complex and may take. The clinical diagnosis of PCA was carried out in accordance with the clinical criteria provided by Crutch et al [4] and was retrospectively confirmed by the revised 2017 criteria [8]: progressive decline in visual abilities, relatively intact memory, and language functions in the early stages and atrophy of posterior regions of the brain [4]. All visual deficits were explained by cognitive and non-ophthalmic impairments. Cerebrospinal fluid (CSF) samples were obtained by lumbar puncture from all patients. CSF levels of total Tau (T-Tau), phospho Tau (p-Tau), Aβ42, and Aβ40 were measured
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