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A Frontotemporal Family Bridge
Arch Neurol. 2004;61:318.
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| Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings. |
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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 17linked 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
Not all dementias linked to chromosome 17 have tau mutations; probably only about 50% do. Approximately 25% to 40% of FTD is familial, and approximately 10% to 30% of patients with a positive family history have a mutation in tau.7 None of the sporadic . . . [Full Text of this Article]
Andrew Kertesz, MD, FRCPC
Department of Clinical Neurological Sciences St. Joseph's Hospital 268 Grosvenor St London, Ontario Canada N6A 4V2 (e-mail: andrew.kertesz@sjhc.london.on.ca)
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