Brain, Vol. 118, No. 1, 1-13, 1995
© 1995 Oxford University Press
research-article |
Progressive prosopagnosia associated with selective right temporal lobe atrophy
A new syndrome?
1MRC Applied Psychology Unit Cambridge 2Addenbrooke's NHS Trust, Fulbourn Hospital Cambridge, UK 3Department of Radiology, Addenbrooke's Hospital Cambridge, UK 4University of Cambridge Neurology unit, Addenbrooke's Hospital Cambridge, UK
Correspondence to:
John R. Hodges, University Neurology unit, Addenbrooke's Hospital, Hills Rd, Cambridge CB2 2QQ, UK or Jonathan J. Evans, MRC Applied Psychology Unit, 15 Chaucer Road, Cambridge CB2 2EF, UK
V.H., a 68-year-old right-handed woman, presented with a progressive deterioration in her ability to recognize faces of familiar people, including friends and relatives. Neuro-psychological testing on two occasions separated by 9 months indicated no deterioration in general intellectual ability from estimates of her pre-morbid IQ (in the high average range), and little or no change in memory, language, perceptual or executive functioning. She is severely impaired on tests of face recognition and has shown some deterioration between testing occasions. In contrast, face perception skills, including emotional expression analysis, appear to be intact. Her knowledge of people from names was originally much better than from faces, but clearly declined on follow-up. This progression is discussed in the context of contemporary models of face processing: we suggest that her prosopagnosic deficit began as a modality-specific disorder which has progressed to a cross-modality loss of person-based semantic knowledge. In addition, she shows a striking dissociation between her ability to recognize faces and unique exemplars from other categories, such as buildings and flowers, which confirms the hypothesis that face processing is indeed special. Scanning by SPECT and MRI revealed selective hypoperfusion and atrophy, respectively, of the anterior part of the right temporal lobe. In recent years there have been a number of descriptions of progressive fluent aphasia resulting from atrophy of the left temporal lobe. This case appears to represent a corresponding degenerative process affecting the right temporal lobe.
prosopagnosia; temporal lobe; semantic knowledge; dementia; material specific memory
Received May 16, 1994. Revised August 30, 1994. Accepted September 27, 1994.
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