Brain, Vol. 115, No. 6, 1783-1806, 1992
© 1992 Guarantors of Brain
research-article |
SEMANTIC DEMENTIA
PROGRESSIVE FLUENT APHASIA WITH TEMPORAL LOBE ATROPHY
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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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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