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Brain, Vol. 115, No. 6, 1783-1806, 1992
© 1992 Guarantors of Brain


research-article

SEMANTIC DEMENTIA

PROGRESSIVE FLUENT APHASIA WITH TEMPORAL LOBE ATROPHY

JOHN R. HODGES1, KARALYN PATTERSON2, SUSAN OXBURY3 and ELAINE FUNNELL4

1University of Cambridge Clinical School, Addenbrooke's Hospital London, UK 2MRC Applied Psychology Unit, Cambridge London, UK 3Department of Neuropsychology, Radcliffe Infirmary, Oxford London, UK 4Department of Psychology, Royal Holloway College, University of London London, UK

Correspondence to: Correspondence to: Dr J. R. Hodges, Department of Neurology, Addenbrooke's Hospital, Hills Road, Cambridge CB2 2QQ, UK.

We report five patients with a stereotyped clinical syndrome characterized by fluent dysphasia with severe anomia, reduced vocabulary and promment impairment of single-word comprehension, progressing to a stage of virtually complete dissolution of the semantic components of language. A marked reduction in the ability to generate exemplars from restricted semantic categories (e.g. animals, vehicles, etc.) was a consistent and early feature. Tests of semantic memory demonstrated a radically impoverished knowledge about a range of living and man-made items. In contrast, phonology and grammar of spoken language were largely preserved, as was comprehension of complex syntactic commands. Reading showed a pattern of surface dyslexia. Autobiographical and day-to-day (episodic) memory were relatively retained. Non-verbal memory, perceptual and visuospatial abilities were also strikingly preserved. In some cases, behavioural and personality changes may supervene; one patient developed features of the Kluver-Bucy Syndrome. Radiological investigations have shown marked focal temporal atrophy in all five patients, and functional imaging by single positron emission tomography and positron emission tomography (one case) have implicated the dominant temporal lobe in all five. In the older literature, such cases would have been subsumed under the rubric of Pick's disease. Others have been included in series with progressive aphasia. We propose the term semantic dementia, first coined by Snowden et al. (1989), to designate this clinical syndrome.

Received April 6, 1992. Accepted May 13, 1992.


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NeurologyHome page
H. J. Rosen, K. M. Hartikainen, W. Jagust, J. H. Kramer, B. R. Reed, J. L. Cummings, K. Boone, W. Ellis, C. Miller, and B. L. Miller
Utility of clinical criteria in differentiating frontotemporal lobar degeneration (FTLD) from AD
Neurology, June 11, 2002; 58(11): 1608 - 1615.
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J. Neurol. Neurosurg. PsychiatryHome page
M D Greicius, M D Geschwind, and B L Miller
Presenile dementia syndromes: an update on taxonomy and diagnosis
J. Neurol. Neurosurg. Psychiatry, June 1, 2002; 72(6): 691 - 700.
[Abstract] [Full Text] [PDF]


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BrainHome page
B. Desgranges, J.-C. Baron, C. Lalevee, B. Giffard, F. Viader, V. de la Sayette, and F. Eustache
The neural substrates of episodic memory impairment in Alzheimer's disease as revealed by FDG-PET: relationship to degree of deterioration
Brain, May 1, 2002; 125(5): 1116 - 1124.
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BrainHome page
S. M. Pickering-Brown, A. M. T. Richardson, J. S. Snowden, A. M McDonagh, A. Burns, W. Braude, M. Baker, W.-K. Liu, S.-H. Yen, J. Hardy, et al.
Inherited frontotemporal dementia in nine British families associated with intronic mutations in the tau gene
Brain, April 1, 2002; 125(4): 732 - 751.
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NeurologyHome page
H. J. Rosen, M. L. Gorno-Tempini, W. P. Goldman, R. J. Perry, N. Schuff, M. Weiner, R. Feiwell, J. H. Kramer, and B. L. Miller
Patterns of brain atrophy in frontotemporal dementia and semantic dementia
Neurology, January 22, 2002; 58(2): 198 - 208.
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AM J ALZHEIMERS DIS OTHER DEMENHome page
A. Kertesz and D. G. Munoz
Primary progressive aphasia: A review of the neourobiology of a common presentation of Pick complex
American Journal of Alzheimer's Disease and Other Dementias, January 1, 2002; 17(1): 30 - 36.
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NeurologyHome page
C. J. Galton, K. Patterson, K. Graham, M.A. Lambon-Ralph, G. Williams, N. Antoun, B.J. Sahakian, and J.R. Hodges
Differing patterns of temporal atrophy in Alzheimer's disease and semantic dementia
Neurology, July 24, 2001; 57(2): 216 - 225.
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NeurologyHome page
M. N. Rossor
Pick's disease: A clinical overview
Neurology, June 12, 2001; 56(90004): S3 - 5.
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NeurologyHome page
J. R. Hodges
Frontotemporal dementia (Pick's disease): Clinical features and assessment
Neurology, June 12, 2001; 56(90004): S6 - 10.
[Abstract] [Full Text] [PDF]


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NeurologyHome page
P. Mychack, J. H. Kramer, K. B. Boone, and B. L. Miller
The influence of right frontotemporal dysfunction on social behavior in frontotemporal dementia
Neurology, June 12, 2001; 56(90004): S11 - 15.
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NeurologyHome page
R. J. Perry and B. L. Miller
Behavior and treatment in frontotemporal dementia
Neurology, June 12, 2001; 56(90004): S46 - 51.
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J. Neurol. Neurosurg. PsychiatryHome page
J S Snowden, D Bathgate, A Varma, A Blackshaw, Z C Gibbons, and D Neary
Distinct behavioural profiles in frontotemporal dementia and semantic dementia
J. Neurol. Neurosurg. Psychiatry, March 1, 2001; 70(3): 323 - 332.
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J. Neurol. Neurosurg. PsychiatryHome page
J Calderon, R J Perry, S W Erzinclioglu, G E Berrios, T R Dening, and J R Hodges
Perception, attention, and working memory are disproportionately impaired in dementia with Lewy bodies compared with Alzheimer's disease
J. Neurol. Neurosurg. Psychiatry, February 1, 2001; 70(2): 157 - 164.
[Abstract] [Full Text] [PDF]



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