Authors:

Galton, C.J., PATTERSON, K., Xuereb, J.H. & HODGES, J.R.

Reference:

Year of publication:

2000

CBU number:

3915

Abstract:

There has been increasing awareness that some slowly progressive focal cortical syndromes can be the presenting features of Alzheimer's disease (AD), but pathological evidence has been sparse. This clinico-pathological series presents our experience with pathologically proven atypical as well as typical AD presentations. We report and compare four patterns of resentation; (1) a typical pattern with initial amnestic syndrome (N=4 cases), (2) progressive visual dysfunction (N=1), (3) progressive biparietal syndrome (N=2) and (4) progressive aphasia (N=6). The aphasic presentations include both fluent and non-fluent aphasic syndromes. The neuropsychological profiles and neuroimaging clearly reflect the presenting clinical features, and show a close relationship to the distribution of pathology in these cases. Of note was the sparing of medial temporal structures (hippocampus and/or entorhinal cortex) in several aphasic cases and the severe occipito-parietal involvement in those with prominent visuospatial disorders at presentations. The recognition of atypical presentations of AD is important when attempting to make an early accurate premorbid diagnosis of neurodegenerative disease.