Frontotemporal dementia and primary progressive aphasia, a review

Abstract

Frontotemporal dementias are neurodegenerative diseases in which symptoms of frontal and/or temporal lobe disease are the first signs of the illness, and as the diseases progress, they resemble a focal left hemisphere process such as stroke or traumatic brain injury, even more than a neurodegenerative disease. Over time, some patients develop a more generalized dementia. Four clinical subtypes characterize the predominant presentations of this illness: behavioral or frontal variant FTD, progressive nonfluent aphasia, semantic dementia, and logopenic primary progressive aphasia. These clinical variants correlate with regional patterns of atrophy on brain imaging studies such as MRI and PET scanning, as well as with biochemical and molecular genetic variants of the disorder. The treatment is as yet only symptomatic, but advances in molecular genetics promise new therapies.

Keywords: FTD; PPA; behavior variant or frontal variant FTD; pick’s disease; progressive nonfluent aphasia.

Figure 1

Personal patient with progressive non-fluent aphasia. Notes: MRI coronal section shows focal left temporal atrophy (A). PET shows left frontotemporal hypometabolism (B). Abbreviations: MRI, magnetic resonance imaging; PET, positron emission tomography.