Abstract
Posterior cortical atrophy (PCA) is an uncommon presentation of Alzheimer's disease (AD), characterised by prevalent anatomo-functional involvement of posterior cortical areas. Accordingly, the main clinical features at onset are disorders of high-order visual processing, such as alexia and impairments of visuo-spatial and visuo-constructional abilities. The clinical features in the early stages of disease are variable, and they have been suggested to stem from prevalent ventral or dorsal brain pathology, and/or asymmetric hemispheric involvement. With disease progression, these differences tend to blur with the increasing severity of neuropsychological dysfunction. We report two PCA patients showing different patterns of reading impairment (respectively, letter-by-letter reading and neglect dyslexia). A follow-up study suggested that the qualitative features of alexia remain distinctive with disease evolution. In addition, single photon emission tomography (SPECT) studies revealed different patterns of hypoperfusion, consistent with the alexia types. A careful reading assessment can provide important insights to the pattern of progression of the disease in patients with PCA up to the late stages of the pathology.
Highlights
Posterior Cortical Atrophy (PCA) is an uncommon neurodegenerative disorder, which is due to progressive dysfunction of the retrorolandic associative cortex
PCA is characterised by impairments of high-order visual processing [8,20,30], and the course of the disease is variable in terms of progression of neuropsychological deficits
This study aims at highlighting the clinical evolution of different types of alexia in two PCA patients, one with a neuropsychological picture compatible with an initial and unilateral involvement of the ventral areas, and the other with features of the dorsal variant and a prominent involvement of right hemisphere
Summary
Posterior Cortical Atrophy (PCA) is an uncommon neurodegenerative disorder, which is due to progressive dysfunction of the retrorolandic associative cortex. This study aims at highlighting the clinical evolution of different types of alexia in two PCA patients, one with a neuropsychological picture compatible with an initial and unilateral involvement (left) of the ventral areas, and the other with features of the dorsal variant and a prominent involvement of right hemisphere. They underwent examination, respectively, at two and three stages in the course of their illness and were submitted to MRI and regional cerebral blood flow (rCBF) SPECT studies
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