| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
|
|
|
|||||||
|
|||||||||
From the University Department of Neurology (Dr. Hodges and R. Davies) and University Department of Pathology (R. Davies and Dr. Xuereb), Addenbrooke’s Hospital, Cambridge; MRC Cognition and Brain Sciences Unit (Dr. Hodges), Cambridge, UK; Centre for Education and Research on Ageing (Dr. Kril), Concord Hospital and University of Sydney, New South Wales; and Prince of Wales Medical Research Institute (Dr. Halliday), the University of New South Wales, Randwick, New South Wales, Australia.
Address correspondence and reprint requests to Professor John Hodges, MRC Cognition and Brain Sciences Unit, 15 Chaucer Rd., Cambridge CB2 2EF, UK; e-mail: john.hodges{at}mrc-cbu.cam.ac.uk
Objectives: To establish survival in patients with pathologically confirmed frontotemporal dementia (FTD) and to determine whether clinical or pathologic subtype affects prognosis.
Methods: The authors reviewed the presenting clinical features of 61 patients with dementia and pathologically confirmed FTD studied in Sydney (n = 31) and Cambridge (n = 30) over a 10-year period. Data were available on time of symptom onset, diagnosis, institutionalization, and death. Cases were classified pathologically as tau-positive and tau-negative.
Results: Of the 61 patients with FTD, 26 presented with frontal variant (fvFTD), 9 with semantic dementia, 8 with progressive nonfluent aphasia (PNFA), 9 with associated motor neuron disease (FTD-MND), and 9 with corticobasal degeneration features. There was no difference between the groups in age at symptom onset (overall mean 58.5 ± 7.8 years), but at diagnosis the PNFA (68.3 ± 2.7) group was significantly older than the fvFTD (59.9 ± 7.4) and FTD-MND (57.7 ± 7.9) groups. The median survival from symptom onset and from diagnosis was 6 ± 1.1 years (95% CI) for fvFTD and 3 ± 0.4 years for FTD-MND. Survival across subgroups was equivalent except for the FTD-MND group, which had significantly shorter survival. Cases with tau-positive pathology had an older age at onset and a significantly better prognosis: median survival 9.0 ± 0.9 years vs 5.0 ± 1.1 years.
Conclusions: FTD is a malignant disorder with limited life expectancy. FTD-MND has the shortest duration both before and after diagnosis. Tau-positivity is associated with a more slowly progressive form of FTD.
This article has been cited by other articles:
|
|
 |
|
  B. Garcin, P. Lillo, M. Hornberger, O. Piguet, K. Dawson SRN, P. J. Nestor, and J. R. Hodges Determinants of survival in behavioral variant frontotemporal dementia Neurology, November 17, 2009; 73(20): 1656 - 1661. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 W. T. Hu, H. Seelaar, K. A. Josephs, D. S. Knopman, B. F. Boeve, E. J. Sorenson, L. McCluskey, L. Elman, H. J. Schelhaas, J. E. Parisi, et al. Survival Profiles of Patients With Frontotemporal Dementia and Motor Neuron Disease Arch Neurol, November 1, 2009; 66(11): 1359 - 1364. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. R. Hodges, J. Mitchell, K. Dawson, M. G. Spillantini, J. H. Xuereb, P. McMonagle, P. J. Nestor, and K. Patterson Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases Brain, October 5, 2009; (2009) awp248v1. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
C. M. Kipps, J. R. Hodges, T. D. Fryer, and P. J. Nestor Combined magnetic resonance imaging and positron emission tomography brain imaging in behavioural variant frontotemporal degeneration: refining the clinical phenotype Brain, September 1, 2009; 132(9): 2566 - 2578. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. Czarnecki, J. R. Duffy, C. R. Nehl, S. A. Cross, J. R. Molano, C. R. Jack Jr, M. M. Shiung, K. A. Josephs, and B. F. Boeve Very Early Semantic Dementia With Progressive Temporal Lobe Atrophy: An 8-Year Longitudinal Study Arch Neurol, December 1, 2008; 65(12): 1659 - 1663. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. D. Rohrer, E. McNaught, J. Foster, S. L. Clegg, J. Barnes, R. Omar, E. K. Warrington, M. N. Rossor, J. D. Warren, and N. C. Fox Tracking progression in frontotemporal lobar degeneration: Serial MRI in semantic dementia Neurology, October 28, 2008; 71(18): 1445 - 1451. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. B. Frisoni and J. L. Whitwell How fast will it go, doc?: New tools for an old question from patients with Alzheimer disease Neurology, June 3, 2008; 70(23): 2194 - 2195. [Full Text] [PDF]
|
|
|
|
|
|
J. Beck, J. D. Rohrer, T. Campbell, A. Isaacs, K. E. Morrison, E. F. Goodall, E. K. Warrington, J. Stevens, T. Revesz, J. Holton, et al. A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series Brain, March 1, 2008; 131(3): 706 - 720. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 S X Xie, M S Forman, J Farmer, P Moore, Y Wang, X Wang, C M Clark, H B Coslett, A Chatterjee, S E Arnold, et al. Factors associated with survival probability in autopsy-proven frontotemporal lobar degeneration J. Neurol. Neurosurg. Psychiatry, February 1, 2008; 79(2): 126 - 129. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
W. W. Seeley, R. Crawford, K. Rascovsky, J. H. Kramer, M. Weiner, B. L. Miller, and M. L. Gorno-Tempini Frontal Paralimbic Network Atrophy in Very Mild Behavioral Variant Frontotemporal Dementia Arch Neurol, February 1, 2008; 65(2): 249 - 255. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 R. J. Caselli and R. Yaari Medical Management of Frontotemporal Dementia American Journal of Alzheimer's Disease and Other Dementias, January 1, 2008; 22(6): 489 - 498. [Abstract] [PDF]
|
|
|
|
|
|
S. Alladi, J. Xuereb, T. Bak, P. Nestor, J. Knibb, K. Patterson, and J. R. Hodges Focal cortical presentations of Alzheimer's disease Brain, October 1, 2007; 130(10): 2636 - 2645. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Grossman, E. M. Wood, P. Moore, M. Neumann, L. Kwong, M. S. Forman, C. M. Clark, L. F. McCluskey, B. L. Miller, V. M.-Y. Lee, et al. TDP-43 Pathologic Lesions and Clinical Phenotype in Frontotemporal Lobar Degeneration With Ubiquitin-Positive Inclusions Arch Neurol, October 1, 2007; 64(10): 1449 - 1454. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
H. Seelaar, H. Jurgen Schelhaas, A. Azmani, B. Kusters, S. Rosso, D. Majoor-Krakauer, M. C. de Rijik, P. Rizzu, M. ten Brummelhuis, P. A. van Doorn, et al. TDP-43 pathology in familial frontotemporal dementia and motor neuron disease without Progranulin mutations Brain, May 1, 2007; 130(5): 1375 - 1385. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
G. D. Rabinovici, A. J. Furst, J. P. O'Neil, C. A. Racine, E. C. Mormino, S. L. Baker, S. Chetty, P. Patel, T. A. Pagliaro, W. E. Klunk, et al. 11C-PIB PET imaging in Alzheimer disease and frontotemporal lobar degeneration Neurology, April 10, 2007; 68(15): 1205 - 1212. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
I. L. Ber, E. Guedj, A. Gabelle, P. Verpillat, M. Volteau, C. Thomas-Anterion, M. Decousus, D. Hannequin, P. Vera, L. Lacomblez, et al. Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia. Brain, November 1, 2006; 129(Pt 11): 3051 - 3065. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. R. Davies, C. M. Kipps, J. Mitchell, J. J. Kril, G. M. Halliday, and J. R. Hodges Progression in Frontotemporal Dementia: Identifying a Benign Behavioral Variant by Magnetic Resonance Imaging Arch Neurol, November 1, 2006; 63(11): 1627 - 1631. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. D. Roberson, J. H. Hesse, K. D. Rose, H. Slama, J. K. Johnson, K. Yaffe, M. S. Forman, C. A. Miller, J. Q. Trojanowski, J. H. Kramer, et al. Frontotemporal dementia progresses to death faster than Alzheimer disease Neurology, September 13, 2005; 65(5): 719 - 725. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. M. Lipton, K. A. Ohman, K. B. Womack, L. S. Hynan, E. T. Ninman, and L. H. Lacritz Subscores of the FAB differentiate frontotemporal lobar degeneration from AD Neurology, September 13, 2005; 65(5): 726 - 731. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
B. Borroni, D. Yancopoulou, M. Tsutsui, A. Padovani, S. J. Sawcer, J. R. Hodges, and M. G. Spillantini Association Between Tau H2 Haplotype and Age at Onset in Frontotemporal Dementia Arch Neurol, September 1, 2005; 62(9): 1419 - 1422. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
R. R. Davies, J. R. Hodges, J. J. Kril, K. Patterson, G. M. Halliday, and J. H. Xuereb The pathological basis of semantic dementia Brain, September 1, 2005; 128(9): 1984 - 1995. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Kertesz, P. McMonagle, M. Blair, W. Davidson, and D. G. Munoz The evolution and pathology of frontotemporal dementia Brain, September 1, 2005; 128(9): 1996 - 2005. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. A. Josephs, D. S. Knopman, J. L. Whitwell, B. F. Boeve, J. E. Parisi, R. C. Petersen, and D. W. Dickson Survival in two variants of tau-negative frontotemporal lobar degeneration: FTLD-U vs FTLD-MND Neurology, August 23, 2005; 65(4): 645 - 647. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
K. Rascovsky, D. P. Salmon, A. M. Lipton, J. B. Leverenz, C. DeCarli, W. J. Jagust, C. M. Clark, M. F. Mendez, D. F. Tang-Wai, N. R. Graff-Radford, et al. Rate of progression differs in frontotemporal dementia and Alzheimer disease Neurology, August 9, 2005; 65(3): 397 - 403. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
J. K. Johnson, J. Diehl, M. F. Mendez, J. Neuhaus, J. S. Shapira, M. Forman, D. J. Chute, E. D. Roberson, C. Pace-Savitsky, M. Neumann, et al. Frontotemporal Lobar Degeneration: Demographic Characteristics of 353 Patients Arch Neurol, June 1, 2005; 62(6): 925 - 930. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M Grossman and P Moore A longitudinal study of sentence comprehension difficulty in primary progressive aphasia J. Neurol. Neurosurg. Psychiatry, May 1, 2005; 76(5): 644 - 649. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
A. Graham, R. Davies, J. Xuereb, G. Halliday, J. Kril, H. Creasey, K. Graham, and J. Hodges Pathologically proven frontotemporal dementia presenting with severe amnesia Brain, March 1, 2005; 128(3): 597 - 605. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
Y. Jeong, K. C. Park, S. S. Cho, E. J. Kim, S. J. Kang, S. E. Kim, E. Kang, and D. L. Na Pattern of glucose hypometabolism in frontotemporal dementia with motor neuron disease Neurology, February 22, 2005; 64(4): 734 - 736. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
M. Broe, J. Kril, and G. M. Halliday Astrocytic degeneration relates to the severity of disease in frontotemporal dementia Brain, October 1, 2004; 127(10): 2214 - 2220. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
D. S. Knopman, R. C. Petersen, S. D. Edland, R. H. Cha, and W. A. Rocca The incidence of frontotemporal lobar degeneration in Rochester, Minnesota, 1990 through 1994 Neurology, February 10, 2004; 62(3): 506 - 508. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH | TABLE OF CONTENTS |
