NON-ALZHEIMER FRONTOTEMPORAL DEGENERATIVE DEMENTIA - A NEUROBEHAVIORAL AND PATHOLOGICAL-STUDY

In contrast to Alzheimer's disease (AD), which predominantly affects t he limbic system and association neocortex, degenerative dementias hav e been identified that show greatest damage in frontal and temporal ne ocortex. This ''lobar atrophy'' was first described by Arnold Pick ove r 100 years ago. We present four autopsy-documented, non-AD cases of f ronto-temporal degeneration (FTD), each with neurobehavioral assessmen t. Three of the patients presented with personality change and the fou rth with non-fluent aphasia; memory was initially preserved in all, an d only later did dementia become evident. At autopsy, the hippocampi w ere relatively spared in all cases. One patient had classic Pick-body temporal lobe Pick's disease (PD), 2 had frontal lobe-predominant atro phy with severe degeneration of caudate and substantia nigra (so-calle d ''generalized Pick's''), and the fourth had temporal lobe degenerati on without Pick bodies or cells. We propose that subgroups of FTD be c ategorized both by microscopic features and gross patterns of atrophy, and that subgroups of FTD be kept separate for neurochemical and gene tic studies. Neurobehavioral and neuropsychological evaluation early i n the course of unusual degenerative dementia can aid in distinguishin g FTD cases from AD patients for clinical and drug studies.