Frontotemporal Dementia -- March 2005

Andrew Kertesz, MD, FRCP(C), Director of Cognitive Neurology, St. Joseph’s Health Center, University of Western Ontario, London, ON.
David Munoz, MD, FRCP(C), Department of Pathology, St. Michael’s Hospital, University of Toronto, Toronto, ON.

Frontotemporal dementia (FTD or Pick’s disease) is a relatively common but underdiagnosed form of presenile dementia. Estimated prevalence is 20% of dementias and 50% of dementia in patients under age 65. Common presentations are disinhibition with indifference; progressive aphasia; semantic dementia; unexplained falls, vertical gaze palsy, and dysarthria; and dementia with motor neuron disease. Neuroimaging is essential to exclude a slow tumour. Tau mutations are found in some families. Atypical neuroleptics and antidepressants can effectively treat some of the characteristics of FTD.

Key words: frontotemporal dementia, Pick’s disease, primary progressive aphasia, corticobasal degeneration, progressive supranuclear palsy.