Posterior cortical atrophy. Two case reports and a review of the literature

We present two cases of progressive early-onset dementia with apraxia and v isuospatial disability as initial manifestations. In the later stages of th e illness Gerstmann's and Balint's syndromes developed. Structural neuroima ging demonstrated parieto-occipital atrophy and functional imaging revealed bilateral hypometabolism and hypoperfusion in these areas. These cases ful fil the diagnostic criteria of posterior cortical atrophy (PCA). Frontal lo be involvement became evident as the disease progressed. Alzheimer's diseas e also typically features this anterior spread and possibly this is the und erlying pathological substrate for this clinical syndrome, although definit e pathology is lacking. In this report, we describe longitudinal evolution in these two cases of PCA. (C) 2001 Elsevier Science B.V. All rights reserv ed.