Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases Export

@article{citeulike:5915731, abstract = {A great deal has been written about cognitive aspects of semantic dementia but little is known about the demography or prognosis. We describe these features in a consecutive series of 100 patients seen over a 17-year period; all cases were assessed and followed up in a specialist clinic. The mean age at diagnosis was 64.2 ({+/-}7.1) range 40-79 years, but 46 presented after age 65 and 7 after 75; a higher proportion than the existing literature might predict. Fifteen had a first-degree relative with dementia, but in seven this was almost certainly unrelated. Only two had relatives with young-onset dementia. There were no families with more than two affected members. The familial rate was estimated at between 2\% and 7\% (95\% confidence interval 0-12\%). Kaplan-Meier analyses indicated a 50\% survival of 12.8 years (95\% confidence interval 11.9-13.7); a more benign course than suggested by neuropathologically based studies. We were unable to identify any factors influencing survival. Of the 100, 34 have died, with pathological confirmation in 24; 18 had frontotemporal lobar degeneration with ubiquitin-positive inclusions (13 of 13 confirmed TAR DNA binding protein-43 positive), and 3 had classic tau-positive Pick bodies and 3 had Alzheimer's pathology. The age at diagnosis or death across the pathological subgroups was equivalent. Although semantic dementia has a strong statistical association with ubiquitin-positive pathology, it does not have the signature of familial frontotemporal lobar degeneration with ubiquitin-positive inclusions, notably the presence of intranuclear lentiform TAR DNA binding protein-43 inclusions. The age of onset is older than predicted and the course more slowly progressive than suggested by earlier studies of small groups of subjects. 10.1093/brain/awp248}, author = {Hodges, John R. and Mitchell, Jo and Dawson, Kate and Spillantini, Maria G. and Xuereb, John H. and McMonagle, Paul and Nestor, Peter J. and Patterson, Karalyn}, citeulike-article-id = {5915731}, citeulike-linkout-0 = {http://dx.doi.org/10.1093/brain/awp248}, citeulike-linkout-1 = {http://brain.oxfordjournals.org/content/awp248v1/.abstract}, citeulike-linkout-2 = {http://brain.oxfordjournals.org/content/awp248v1/.full.pdf}, citeulike-linkout-3 = {http://view.ncbi.nlm.nih.gov/pubmed/19805492}, citeulike-linkout-4 = {http://www.hubmed.org/display.cgi?uids=19805492}, day = {5}, doi = {10.1093/brain/awp248}, issn = {1460-2156}, journal = {Brain}, keywords = {clinical, ftld, semantic\_dementia}, month = {October}, pages = {awp248+}, posted-at = {2009-10-09 12:39:42}, priority = {3}, title = {Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases}, url = {http://dx.doi.org/10.1093/brain/awp248}, year = {2009} }

TY - JOUR ID - citeulike:5915731 L3 - citeulike-article-id:5915731 N2 - A great deal has been written about cognitive aspects of semantic dementia but little is known about the demography or prognosis. We describe these features in a consecutive series of 100 patients seen over a 17-year period; all cases were assessed and followed up in a specialist clinic. The mean age at diagnosis was 64.2 ({+/-}7.1) range 40-79 years, but 46 presented after age 65 and 7 after 75; a higher proportion than the existing literature might predict. Fifteen had a first-degree relative with dementia, but in seven this was almost certainly unrelated. Only two had relatives with young-onset dementia. There were no families with more than two affected members. The familial rate was estimated at between 2% and 7% (95% confidence interval 0-12%). Kaplan-Meier analyses indicated a 50% survival of 12.8 years (95% confidence interval 11.9-13.7); a more benign course than suggested by neuropathologically based studies. We were unable to identify any factors influencing survival. Of the 100, 34 have died, with pathological confirmation in 24; 18 had frontotemporal lobar degeneration with ubiquitin-positive inclusions (13 of 13 confirmed TAR DNA binding protein-43 positive), and 3 had classic tau-positive Pick bodies and 3 had Alzheimer's pathology. The age at diagnosis or death across the pathological subgroups was equivalent. Although semantic dementia has a strong statistical association with ubiquitin-positive pathology, it does not have the signature of familial frontotemporal lobar degeneration with ubiquitin-positive inclusions, notably the presence of intranuclear lentiform TAR DNA binding protein-43 inclusions. The age of onset is older than predicted and the course more slowly progressive than suggested by earlier studies of small groups of subjects. 10.1093/brain/awp248 JF - Brain SN - 1460-2156 TI - Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases SP - awp248 KW - clinical KW - ftld KW - semantic_dementia AU - Hodges, John AU - Mitchell, Jo AU - Dawson, Kate AU - Spillantini, Maria AU - Xuereb, John AU - McMonagle, Paul AU - Nestor, Peter AU - Patterson, Karalyn PY - 2009/10/05/ UR - http://dx.doi.org/10.1093/brain/awp248 ER -