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Frontotemporal lobar degeneration

A consensus on clinical diagnostic criteria

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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				S. J. Banks and S. Weintraub Cognitive Deficits and Reduced Insight in Primary Progressive Aphasia American Journal of Alzheimer's Disease and Other Dementias, August 1, 2008; 23(4): 363 - 371. [Abstract] [PDF]

				W.-J. Moon, H.-J. Kim, H.G. Roh, and S.-H. Han Atrophy Measurement of the Anterior Commissure and Substantia Innominata with 3T High-Resolution MR Imaging: Does the Measurement Differ for Patients with Frontotemporal Lobar Degeneration and Alzheimer Disease and for Healthy Subjects? AJNR Am. J. Neuroradiol., August 1, 2008; 29(7): 1308 - 1313. [Abstract] [Full Text] [PDF]

				A.J. Bastos-Leite, J.P.A. Kuijer, S.A.R.B. Rombouts, E. Sanz-Arigita, E.C. van Straaten, A.A. Gouw, W.M. van der Flier, P. Scheltens, and F. Barkhof Cerebral Blood Flow by Using Pulsed Arterial Spin-Labeling in Elderly Subjects with White Matter Hyperintensities AJNR Am. J. Neuroradiol., August 1, 2008; 29(7): 1296 - 1301. [Abstract] [Full Text] [PDF]

				J. D. Sluimer, W. M. van der Flier, G. B. Karas, N. C. Fox, P. Scheltens, F. Barkhof, and H. Vrenken Whole-Brain Atrophy Rate and Cognitive Decline: Longitudinal MR Study of Memory Clinic Patients Radiology, August 1, 2008; 248(2): 590 - 598. [Abstract] [Full Text] [PDF]

				I. Kahn, J. R. Andrews-Hanna, J. L. Vincent, A. Z. Snyder, and R. L. Buckner Distinct Cortical Anatomy Linked to Subregions of the Medial Temporal Lobe Revealed by Intrinsic Functional Connectivity J Neurophysiol, July 1, 2008; 100(1): 129 - 139. [Abstract] [Full Text] [PDF]

				S. J. Banks and S. Weintraub Neuropsychiatric Symptoms in Behavioral Variant Frontotemporal Dementia and Primary Progressive Aphasia J Geriatr Psychiatry Neurol, June 1, 2008; 21(2): 133 - 141. [Abstract] [PDF]

				S L Miller, E Fenstermacher, J Bates, D Blacker, R A Sperling, and B C Dickerson Hippocampal activation in adults with mild cognitive impairment predicts subsequent cognitive decline J. Neurol. Neurosurg. Psychiatry, June 1, 2008; 79(6): 630 - 635. [Abstract] [Full Text] [PDF]

				A Alberici, L Bocchio, C Geroldi, R Zanardini, C Bonomini, G Bugari, C Iacobello, L Caimi, M Gennarelli, O Zanetti, et al. Serum leptin levels are higher in females affected by frontotemporal lobar degeneration than Alzheimer's disease J. Neurol. Neurosurg. Psychiatry, June 1, 2008; 79(6): 712 - 715. [Abstract] [Full Text] [PDF]

				M. F. Mendez, E. A. Licht, and J. S. Shapira Changes in Dietary or Eating Behavior in Frontotemporal Dementia Versus Alzheimer's Disease American Journal of Alzheimer's Disease and Other Dementias, June 1, 2008; 23(3): 280 - 285. [Abstract] [PDF]

				M. Hoefer, S. C. Allison, G. F. Schauer, J. M. Neuhaus, J. Hall, J. N. Dang, M. W. Weiner, B. L. Miller, and H. J. Rosen Fear conditioning in frontotemporal lobar degeneration and Alzheimer's disease Brain, June 1, 2008; 131(6): 1646 - 1657. [Abstract] [Full Text] [PDF]