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Scientific Commentaries |
Frontotemporal lobar degeneration through loss of progranulin function
1 MRC Laboratory of Molecular Biology Cambridge, UK 2 Centre for Brain Repair, Department of Clinical Neurosciences, University of Cambridge Cambridge, UK
Correspondence to: M. Goedert, MRC Laboratory of Molecular Biology, Cambridge, CB2 2QH, UK E-mail: mg@mrc-lmb.cam.ac.uk
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The striking clinical and pathological features of frontotemporal lobar degeneration (FTLD) have attracted much attention ever since Arnold Pick reported the first disease cases and Alois Alzheimer described the characteristic nerve cell inclusions of Pick's disease. We now know that FTLD is a common cause of dementia, especially in the population below the age of 65 (Neary et al., 2005
). Clinically, it encompasses cases of frontotemporal dementia (FTD), semantic dementia (SD) and primary progressive aphasia (PPA). Of these, FTD is the most common. It is characterized by progressive behavioural and personality disturbances, which evolve gradually into cognitive impairment and dementia. SD is characterized by a loss of conceptual knowledge, whereas in PPA progressive language impairment usually precedes the behavioural symptoms. In some patients, behavioural symptoms are also associated with a parkinsonian syndrome or with motor neuron disease (MND).
Pathologically, FTLD is characterized by the severe degeneration of frontal
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