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Posterior Cortical Atrophy Due to Alzheimer Disease in a Person With Down Syndrome: A Case Report.

Neurology

January 2025

Sant Pau Memory Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau (IIB Sant Pau), Universitat Autònoma de Barcelona.

Objectives: Atypical variants are rare in genetically determined Alzheimer disease (AD). This case describes a patient with Down syndrome-associated Alzheimer disease (DSAD) who presented with symptoms of posterior cortical atrophy (PCA).

Methods: We conducted a clinical and cognitive evaluation, genotyping, determination of AD biomarkers in CSF, structural MRI, [18F]FDG-PET, and tau-PET ([18F]PI2620) scans.

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Brain MRI Lesions in Alexia Without Agraphia: A Case-Control Study.

J Neuroophthalmol

November 2024

Kellogg Eye Center and Department of Ophthalmology and Visual Sciences (JNS, JDT, SK), University of Michigan, Ann Arbor, Michigan; Departments of Radiology (AS) and Neurology (JDT, SK), University of Michigan, Ann Arbor, Michigan; and Department of Ophthalmology (RDW), Medical College of Wisconsin, Milwaukee, Wisconsin.

Background: Alexia without agraphia (AWA) is an acquired reading disturbance associated with left posterior cerebral artery (PCA) infarction. Based on autopsy and neuroimaging, there are two explanations for its pathogenesis: a visual cortex-language cortex disconnection and a visual word-form agnosia. Our goal was to discover if more refined brain imaging in a case-control study would provide further imaging support for either of these hypotheses.

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Article Synopsis
  • Anosognosia for visual deficits refers to cases where individuals are unaware of significant issues with their vision, challenging the belief that we fully understand our own experiences.
  • This phenomenon can occur in conditions such as total blindness, brain lesions, or eye diseases, illustrating its widespread nature.
  • The authors propose a framework highlighting that recognizing a visual deficit requires forming normal vision expectations, comparing them to actual visual input, and assessing any discrepancies at a higher cognitive level; failures in any of these steps can result in a lack of awareness of the deficits.
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Article Synopsis
  • Optic Aphasia (OA) and Associative Visual Agnosia (AVA) are disorders that affect the ability to name objects presented visually, with OA allowing some access to semantic knowledge and AVA not.
  • The case study examines AA, an 81-year-old woman with a left occipital lesion and damage to the splenial pathway, highlighting her challenges in identifying visual stimuli.
  • The findings indicate that while AA has difficulties, there is still some semantic access possible from visual input, supporting the theory of visuo-verbal disconnection related to OA.
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Optic aphasia is a rare neurological disorder that affects the visual-semantic ability of patients with normal vision and is caused by a lesion in the left occipital lobe. The signs and symptoms of optic aphasia are similar to those of associative visual agnosia, where patients have difficulty recognizing objects both in shape and function, resulting in challenges performing daily tasks. The transformation to optic aphasia or associative visual agnosia is closely related to the degree of damage to the corpus callosum, with some studies hypothetically suggesting that complete damage to the corpus callosum leads to optic aphasia, whereas incomplete damage causes associative visual agnosia.

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