__Abstract__ In 1892, Arnold Pick described the first patients with a clinical syndrome that is currently named frontotemporal dementia (FTD). He emphasised the focal aspect of cortical atrophy in his patients, to this day the hallmark of this disorder. Following a detailed description of the neuropathological changes by Alois Alzheimer in 1911, including the argyrophilic neuronal inclusions later known as Pick bodies, the term Pick’s disease was introduced in 1926. Over the years, many different names have been used to describe this clinical and pathological entity: frontal lobe dementia, dementia of non-Alzheimer type, dementia of frontal lobe type, Pick’s disease, and others. In 1994, the term FTD was introduced to describe the clinical syndrome associated with focal frontotemporal degeneration, with semantic dementia and primary progressive aphasia being recognised as clinical variants of FTD. Typical features of this syndrome, which often presents with a presenile onset, are progressive behavioural changes and language disturbances. FTD can be caused by a number of neuropathological substrates, including Pick’s disease, which is currently defined by the presence of argyrophilic Pick bodies. In 1994, a genetic-epidemiological study on FTD was started at the Erasmus MC of Rotterdam. The main research questions addressed in this study are the estimation of the prevalence of FTD in the Netherlands, the clinical aspects of the temporal variant of FTD, and further elucidation of the genetic and other risk factors involved in the aetiology of FTD.