With or without FUS, it is the anatomy that dictates the dementia phenotype
Open Access
- 27 October 2009
- journal article
- editorial
- Published by Oxford University Press (OUP) in Brain
- Vol. 132 (11) , 2906-2908
Abstract
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).Keywords
This publication has 27 references indexed in Scilit:
- Quantitative Template for Subtyping Primary Progressive AphasiaArchives of Neurology, 2009
- Distinct anatomical subtypes of the behavioural variant of frontotemporal dementia: a cluster analysis studyBrain, 2009
- A new subtype of frontotemporal lobar degeneration with FUS pathologyBrain, 2009
- Neurodegenerative Diseases Target Large-Scale Human Brain NetworksNeuron, 2009
- Update on Recent Molecular and Genetic Advances in Frontotemporal Lobar DegenerationJournal of Neuropathology & Experimental Neurology, 2008
- Alzheimer and frontotemporal pathology in subsets of primary progressive aphasiaAnnals of Neurology, 2008
- Refining Frontotemporal Dementia With Parkinsonism Linked to Chromosome 17Archives of Neurology, 2008
- Frontal Paralimbic Network Atrophy in Very Mild Behavioral Variant Frontotemporal DementiaArchives of Neurology, 2008
- TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosisBiochemical and Biophysical Research Communications, 2006
- Neuropathological stageing of Alzheimer-related changesActa Neuropathologica, 1991