TY - JOUR
T1 - Frontotemporal dementia
AU - Graff-Radford, Neill R.
AU - Woodruff, Bryan K.
N1 - Copyright:
Copyright 2008 Elsevier B.V., All rights reserved.
PY - 2007/2
Y1 - 2007/2
N2 - Frontotemporal dementia (FTD) is an uncommon but important form of degenerative disease. It may make up 50% of dementia cases presenting before age 60. The symptoms are related to the anatomic areas affected. Neary divided the clinical syndromes into "frontotemporal dementia," "progressive nonfluent aphasia," and "semantic dementia." However, the pathology may extend beyond the frontal and temporal lobes and additional symptoms may be found. Although most cases are sporadic, some cases are genetic. The best-known genetic mutation causing FTD is frontotemporal dementia with parkinsonism, linked to the microtubule-associated protein tau on chromosome 17. There are other known genes and chromosome loci related to FTD. The most common pathology found is frontotemporal degeneration with ubiquitin inclusions. In contrast, FTD with Pick bodies is rare. Although there are strategies to help patients and their families, there is no known treatment for the disease.
AB - Frontotemporal dementia (FTD) is an uncommon but important form of degenerative disease. It may make up 50% of dementia cases presenting before age 60. The symptoms are related to the anatomic areas affected. Neary divided the clinical syndromes into "frontotemporal dementia," "progressive nonfluent aphasia," and "semantic dementia." However, the pathology may extend beyond the frontal and temporal lobes and additional symptoms may be found. Although most cases are sporadic, some cases are genetic. The best-known genetic mutation causing FTD is frontotemporal dementia with parkinsonism, linked to the microtubule-associated protein tau on chromosome 17. There are other known genes and chromosome loci related to FTD. The most common pathology found is frontotemporal degeneration with ubiquitin inclusions. In contrast, FTD with Pick bodies is rare. Although there are strategies to help patients and their families, there is no known treatment for the disease.
KW - Frontotemporal dementia
KW - Pick's disease
KW - Primary progressive aphasia
KW - Tau
KW - Ubiquitin
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U2 - 10.1055/s-2006-956755
DO - 10.1055/s-2006-956755
M3 - Review article
C2 - 17226741
AN - SCOPUS:33846934718
SN - 0271-8235
VL - 27
SP - 48
EP - 57
JO - Seminars in Neurology
JF - Seminars in Neurology
IS - 1
ER -