EARLY DETECTION OF FRONTOTEMPORAL DEGENERATION BY CLINICAL-EVALUATION

Although Alzheimer's Disease (AD) is the most common degenerative deme ntia, other neuropathological processes are well known to cause dement ia syndromes. Fronto-temporal degeneration (FTD) is one such entity, a nd often produces a clinical presentation distinct from that of AD. So me FTD cases are shown at autopsy to be classic Pick's Disease, but ot hers defy precise classification at this point because they lack chara cteristic Pick bodies. We describe a case of putative FTD in a 54-year -old man with personality change and complete Kluver-Bucy syndrome. Ne uropsychological evaluation disclosed evidence of extensive frontal sy stem dysfunction, with lesser problems in memory, language, and visuos patial skills. Magnetic resonance imaging studies after 17 months of t he illness were largely nonspecific, but a follow-up scan 16 months la ter revealed bifrontal and bitemporal atrophy with ventricular enlarge ment. Neuromorphometric analysis suggested an increase in cortical atr ophy and ventricular dilation over time. This case emphasizes the valu e of careful clinical evaluation in unusual non-AD degenerative dement ial and suggests that neuroimaging studies may be less sensitive in th e early diagnosis of such cases.