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Scientific Commentary |
With or without FUS, it is the anatomy that dictates the dementia phenotype
Cognitive Neurology and Alzheimer's Disease Centre, Northwestern University, Chicago, IL, USA
sweintraub@northwestern.edu
| The first 150 words of the full text of this article appear below. |
Not so long ago, Alois Alzheimer's dominance over Arnold Pick appeared total and irreversible. Alzheimer's disease was placed at the helm of a global pandemic whilst Pick's disease was declared a rare curiosity and other non-Alzheimer dementias faced the embarrassment of lacking distinctive histopathology. How rapidly things change! We now know that the family of frontotemporal lobar degenerations (FTLDs), which includes Pick's disease, is arguably the most important cause of pre-senescent dementia; that its hereditary forms can be transmitted through mutations in the tau or progranulin (PGRN) genes; and that the abnormal protein accumulations include hyperphosphorylated tau and the TAR DNA binding protein TDP-43 (Arai et al., 2006
; Bigio, 2008; Boeve and Hutton, 2008; Josephs, 2008; Neumann et al., 2006).
Despite this relatively rapid progress over the past 15 years, there still remain FTLD cases either having non-specific ubiquitin deposits or