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NEUROLOGY 1998;51:1546-1554
© 1998 American Academy of Neurology

Frontotemporal lobar degeneration

A consensus on clinical diagnostic criteria
D. Neary, MD, J. S. Snowden, PhD, L. Gustafson, MD, U. Passant, MD, D. Stuss, PhD, S. Black, MD, M. Freedman, MD, A. Kertesz, MD, P. H. Robert, MD, PhD, M. Albert, PhD, K. Boone, PhD, B. L. Miller, MD, J. Cummings, MD and D. F. Benson, MD

From the Manchester Royal Infirmary (Drs. Neary and Snowden), Manchester, UK; the Lund University Hospital (Drs. Gustafson and Passant), Sweden; the Rotman Research Institute (Drs. Stuss, Black, and Freedman) Baycrest Centre for Geriatric Care, Toronto and University of Toronto, Canada; the University of Western Ontario (Dr. Kertesz) London, Canada; the Nice University School of Medicine (Dr. Robert), France; the Massachusetts General Hospital (Dr. Albert), Boston, MA; and the University of California at Los Angeles, School of Medicine (Drs. Boone, Miller, Cummings, and Benson), CA.

Address correspondence and reprint requests to Dr. D. Neary, Department of Neurology, Manchester Royal Infirmary, Manchester M13 9WL, UK.

Objective: To improve clinical recognition and provide research diagnostic criteria for three clinical syndromes associated with frontotemporal lobar degeneration.

Methods: Consensus criteria for the three prototypic syndromes-frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia-were developed by members of an international workshop on frontotemporal lobar degeneration. These criteria build on earlier published clinical diagnostic guidelines for frontotemporal dementia produced by some of the workshop members.

Results: The consensus criteria specify core and supportive features for each of the three prototypic clinical syndromes and provide broad inclusion and exclusion criteria for the generic entity of frontotemporal lobar degeneration. The criteria are presented in lists, and operational definitions for features are provided in the text.

Conclusions: The criteria ought to provide the foundation for research work into the neuropsychology, neuropathology, genetics, molecular biology, and epidemiology of these important clinical disorders that account for a substantial proportion of cases of primary degenerative dementia occurring before the age of 65 years.

Supported by The French Foundation and Baycrest Centre for Geriatric Care. Studies of FTD were supported in part by the Wellcome Trust and a National Institute on Aging Alzheimer's Disease Center grant (AG 10123).

Received March 27, 1998. Accepted in final form August 8, 1998.




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A new subtype of frontotemporal lobar degeneration with FUS pathology
Brain, November 1, 2009; 132(11): 2922 - 2931.
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J. L. Whitwell, S. A. Przybelski, S. D. Weigand, R. J. Ivnik, P. Vemuri, J. L. Gunter, M. L. Senjem, M. M. Shiung, B. F. Boeve, D. S. Knopman, et al.
Distinct anatomical subtypes of the behavioural variant of frontotemporal dementia: a cluster analysis study
Brain, November 1, 2009; 132(11): 2932 - 2946.
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F. Moreno, B. Indakoetxea, M. Barandiaran, A. Alzualde, A. Gabilondo, A. Estanga, J. Ruiz, M. Ruibal, A. Bergareche, J. F. Marti-Masso, et al.
"Frontotemporoparietal" dementia: Clinical phenotype associated with the c.709-1G>A PGRN mutation
Neurology, October 27, 2009; 73(17): 1367 - 1374.
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R. G. Miller, C. E. Jackson, E. J. Kasarskis, J. D. England, D. Forshew, W. Johnston, S. Kalra, J. S. Katz, H. Mitsumoto, J. Rosenfeld, et al.
Practice Parameter update: The care of the patient with amyotrophic lateral sclerosis: Multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): Report of the Quality Standards Subcommittee of the American Academy of Neurology
Neurology, October 13, 2009; 73(15): 1227 - 1233.
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C. Goizet, A. Boukhris, D. Maltete, L. Guyant-Marechal, J. Truchetto, E. Mundwiller, S. Hanein, P. Jonveaux, F. Roelens, J. Loureiro, et al.
SPG15 is the second most common cause of hereditary spastic paraplegia with thin corpus callosum
Neurology, October 6, 2009; 73(14): 1111 - 1119.
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J. A. Knibb, A. M. Woollams, J. R. Hodges, and K. Patterson
Making sense of progressive non-fluent aphasia: an analysis of conversational speech
Brain, October 1, 2009; 132(10): 2734 - 2746.
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J. L. Whitwell, C. R. Jack Jr, B. F. Boeve, M. L. Senjem, M. Baker, R. J. Ivnik, D. S. Knopman, Z. K. Wszolek, R. C. Petersen, R. Rademakers, et al.
Atrophy patterns in IVS10+16, IVS10+3, N279K, S305N, P301L, and V337M MAPT mutations
Neurology, September 29, 2009; 73(13): 1058 - 1065.
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J. Suarez, M. C. Tartaglia, P. Vitali, A. Erbetta, J. Neuhaus, V. Laluz, and B. L. Miller
CHARACTERIZING RADIOLOGY REPORTS IN PATIENTS WITH FRONTOTEMPORAL DEMENTIA
Neurology, September 29, 2009; 73(13): 1073 - 1074.
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J.C.L. Looi, L. Svensson, O. Lindberg, B.B. Zandbelt, P. Ostberg, E. Orndahl, and L.-O. Wahlund
Putaminal Volume in Frontotemporal Lobar Degeneration and Alzheimer Disease: Differential Volumes in Dementia Subtypes and Controls
AJNR Am. J. Neuroradiol., September 1, 2009; 30(8): 1552 - 1560.
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S. Gupta, O. Fiertag, and J. Warner
Rare and unusual dementias
Adv. Psychiatr. Treat., September 1, 2009; 15(5): 364 - 371.
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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.
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Y. Zhang, N. Schuff, A.-T. Du, H. J. Rosen, J. H. Kramer, M. L. Gorno-Tempini, B. L. Miller, and M. W. Weiner
White matter damage in frontotemporal dementia and Alzheimer's disease measured by diffusion MRI
Brain, September 1, 2009; 132(9): 2579 - 2592.
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K. Rascovsky, M. E. Growdon, I. R. Pardo, S. Grossman, and B. L. Miller
'The quicksand of forgetfulness': semantic dementia in One Hundred Years of Solitude
Brain, September 1, 2009; 132(9): 2609 - 2616.
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M. Mesulam, E. Rogalski, C. Wieneke, D. Cobia, A. Rademaker, C. Thompson, and S. Weintraub
Neurology of anomia in the semantic variant of primary progressive aphasia
Brain, September 1, 2009; 132(9): 2553 - 2565.
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J. van der Zee, D. Pirici, T. Van Langenhove, S. Engelborghs, R. Vandenberghe, M. Hoffmann, G. Pusswald, M. Van den Broeck, K. Peeters, M. Mattheijssens, et al.
Clinical heterogeneity in 3 unrelated families linked to VCP p.Arg159His
Neurology, August 25, 2009; 73(8): 626 - 632.
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D. J. Libon, C. McMillan, D. Gunawardena, C. Powers, L. Massimo, A. Khan, B. Morgan, C. Farag, L. Richmond, J. Weinstein, et al.
Neurocognitive contributions to verbal fluency deficits in frontotemporal lobar degeneration
Neurology, August 18, 2009; 73(7): 535 - 542.
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C. E. Krueger, A. C. Bird, M. E. Growdon, J. Y. Jang, B. L. Miller, and J. H. Kramer
Conflict monitoring in early frontotemporal dementia
Neurology, August 4, 2009; 73(5): 349 - 355.
[Abstract] [Full Text] [PDF]


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