Abstract
Posterior cortical atrophy (PCA) is a neurodegenerative syndrome, usually due to Alzheimer's disease. The first symptoms are progressive impairment of visuo spatial (Balint's and Gertsmann's syndromes) or visuo perceptive (visual agnosia, alexia) function. Episodic memory and executive function are spared until later stages. We report two males aged 51 and 55years and three females aged 50, 54 and 56 years, with posterior cortical atrophy. Ophthalmologic study was normal in all. Presenting signs and symptoms were visual ataxia, simultagnosia, agraphia, acalculia, spatial disorientation and unilateral neglect (Balint's and Gerstmann's syndromes). Apperceptive visual agnosia, aphasia, apraxia and alexia were also observed. One female had cortical blindness. Structural images were inconclusive, but PET scan and SPECT disclosed functional impairments in occipitotemporal or occipitoparietal areas.
Highlights
Posterior cortical atrophy (PCA) is a neurodegenerative syndrome, usually due to Alzheimer’s disease
We report two males aged 51 and 55 years and three females aged 50, 54 and 56 years, with posterior cortical atrophy
The topography of metabolic deficits in posterior cortical atrophy (the visual variant of Alzheimer’s disease) with FDG-PET
Summary
2. Ausencia de una patología oftalmológica que explique los síntomas; la agudeza visual es normal, puede existir restricción u otros defectos del campo visual. En 1999 Alberca señalaba que entre los 14 casos de ACP con más de 2 años de evolución (para descartar la forma posterior de la enfermedad de Creuztfeldt Jakob) que habían llegado a la autopsia, 13 correspondían a EA1. Leía en forma muy lenta “le costaba juntar las letras” y tuvo que dejar de manejar por la dificultad en percibir los otros vehículos. Denominó bien colores y objetos, pero con algunos dibujos cometió errores por percepción de detalles aislados: ante el dibujo de un barco dijo “¿una nota musical?” señalando una ventana ovalada; por una mesa dijo “unos pilares” refiriéndose a las patas del mueble.
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