Posterior cortical atrophy. Report of five cases

Posterior conical atrophy (PCA) is a neurodegenerative syndrome, usually due to Alzheimer's disease. The first symptoms are progressive impairment of visuospatial (Balint's and Gertsmann's syndromes) or visuoperceptive (visual agnosia, alexia) function. Episodic memory and executive function are spared until later stages. We report two males aged 51 and 55 years and three females aged 50, 54 and 56 years, with posterior conical atrophy. Ophthalmological 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 a agnosia, aphasia, apraxia and alexia were also observed. One female bad cortical blindness. Structural images were inconclusive, but PET scan and SPECT disclosed functional impairments in occipitotemporal or occipito parietal areas (Rev Med Chile 2009; 137: 1482-7).