Frontotemporal dementia. Review of 63 cases

Background: Frontotemporal dementias (FTD) are neurodegenerative disorders characterized by alterations in behavior affection and language, with relative sparing of episodic memory. There are three major forms of FTD: the frontal or behavioral form, progressive non-fluent aphasia and semantic dementia (that may begin as a fluent progressive aphasia). Aim: To report a retrospective clinical experience of patients with frontotemporal dementia. Material and methods: Review of 3,700 records of neuropsychological assessments of patients with behavioral disturbances, studied between 1981 and 2008. Of these, 63 patients (59% females) complied with the criteria for frontotemporal dementia. Results: There were 47 cases with the frontal variant, four with non-fluent progressive aphasia and six with fluent progressive aphasias (2 evolved to semantic dementia). The mean age of onset was 60 +/- 11 years. There were no familiar cases of FTD. Conclusions. It is clinically difficult to diagnose FTD, since evaluation of attitude or language is required. In addition to structural images, functional images were helpful in some cases, Wit the definitive diagnosis is anatomical (Rev Med Chile 2009; 137: 900-5).