This review ofpharmacotherapyforfrontotemporal lobar degeneration describes the chemical rationale for agents used and reports observed responses to psychotropic medications. Paroxetine addressed anxiety and repetitive, ritualistic behaviors. Depression was resistant to treatment. Valproic acid and quetiapine calmed agitated subjects without exacerbating Parkinsonism. Donepezil has not emerged as a beneficial medication for this group of subjects. Behavioral disturbances can be alleviatedpharmacologically, but further research might determine guidelines for management of this non-Alzheimer's dementia syndrome that could decrease the frequency of adverse drug events.