One of the major discoveries in dementia has been the linkage of a family with disinhibition-dementia-parkinsonism amyotrophy complex to chromosome 17.1 Soon after that, several other families were linked to chromosome 17, many described before, under various names, such as hereditary dysphasic dementia2 and pallido-ponto-nigral degeneration.3 It was soon recognized that all these conditions have clinical and pathological resemblance to sporadic frontotemporal dementia (FTD), primary progressive aphasia, and corticobasal ganglionic degeneration (CBD). A conference about chromosome 17–linked dementias agreed on the apt term frontotemporal dementia with parkinsonism linked to chromosome 17.4 Although Wilhelmsen considered tau the candidate gene, tau mutations were described later.5,6
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