神经病学和神经科学杂志

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A Case of Posterior Cortical Atrophy with Complex Set of Symptoms and Rapid Course

Tondo G, F De Marchi, Terazzi E, Sacchetti M and Cantello R

Posterior Cortical Atrophy (PCA), also called Benson’s syndrome, is a neurodegenerative disease characterized by a progressive occipital and parietal dysfunction, including visuospatial and visuoperceptual disorders, apraxia, alexia, acalculia and language deficit, with relative sparing of memory. We describe a case of PCA with some typical characteristics, such as visual symptoms at the onset, neuroimaging and CSF findings, but unusual for a tumultuous course with a rapidly worsening dementia and for the association with infrequent signs, such as myoclonus, postural tremor and grasping reflex.