October 12 Highlights

Abstract

Tang-Wai et al. report the clinical, genetic, and neuropathologic characteristics of 40 patients with posterior cortical atrophy. Occipito-parietal dysfunction dominates the presentation. Their genetics and histopathology are like that of Alzheimer disease. However, the greatest concentration of neurofibrillary tangles is in extrastriate visual association cortex, not the hippocampus. ⇓ Serial MRI images of a patient with PCA showing occipito-parietal atrophy (large white arrows). see page 1168 ### The differential diagnosis of posterior cortical dementia Posterior cortical dysfunction presented with progressive visuospatial deficits in 27 patients described by Renner et al. These patients have a barrage of aphasias, apraxias, and elements of Balint’s and Gerstmann’s syndromes. Although Alzheimer neuropathology is most common, tauopathic and prion related pathologies occur. This syndrome is clinically distinguishable from other late-life dementias. see page 1175 ### Lost but not forgetting: Posterior cortical Alzheimer disease The accompanying editorial by Charles J. Duffy discusses how Tang-Wai et al. and Renner et al. echo Alois Alzheimer’s description of focal cortical deficits. Phenotypic diversity in Alzheimer disease may betray genotypic heterogeneity or interactions between genetic predispositions and physiologic modulation. In either case, more specific diagnosis may be a further step toward clarifying pathophysiology and individualizing therapy . see page 1148 In a follow-up study on …