Frontotemporal dementia (FTD) is a degenerative disease of the brain. The frontal areas of the brain that are affected by degeneration of neurones and accumulation of tau and other inclusion material control personality, social conduct, speech and language, organization and reasoning. In the early stages of the disease FTD patients present with disturbance of interpersonal conduct, loss of empathy and loss of insight. Later on patients progressively develop memory problems, aphasia and behavioral alterations such as pacing and repetitive stereotyped behaviors (1, 2). Due to the behavioral features of FTD, FTD is often misdiagnosed in its early phases (3). The age of onset of FTD ranges from 25 to 64 years partly depending on the specific underlying gene mutation (4) and men and women are equally at-risk (5). The mean course of FTD is 8 years from the time of diagnosis. The prevalence of FTD is estimated at 5 to 15 cases per 100.000 in the age group of 45 to 64 years in the Netherlands, thus FTD is recognized as the second most common type of presenile dementia in the Netherlands (6). Around 70-80% of cases are sporadic (6), whereas genetic mutations are found in 40% of cases with a positive family history (4, 6). The inheritance is mostly autosomal dominant, implying that children of a patient have 50% risk of receiving either the disease or the normal gene from the affected parent. The symptoms of the disease may vary significantly between affected relatives.

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EMC, Rotterdam, Alzheimer Nederland
Erasmus MC: University Medical Center Rotterdam
M.F. Niermeijer (Martinus) , A. Tibben (Arend)
Erasmus MC: University Medical Center Rotterdam

Riedijk, S. (2009, May 8). Burdening Care: A Study on Informal Caregivers of Frontotemporal Dementia Patients. Retrieved from