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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.

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This case report presents the story of Mr. S, a professional orchestral musician with declining musical sight-reading ability, followed by progressive visuospatial and language deficits. Our novel musical assessment battery revealed deficits in music-reading (musical alexia) and music-writing (musical agraphia), with spared auditory perception and expression.

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Background: There are two types of central auditory disorders due to pathology of the bilateral auditory cortices in adult patients: with residual hearing; with total hearing loss. However, long-term changes of hearing acuity over physical development time are unknown.

Objective: The aim of this case report was to illustrate the pathophysiology of auditory changes in a 1 year-3 months old child who was diagnosed with auditory agnosia as a sequel of herpes encephalitis and later developed cortical deafness during a 36-year follow-up.

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