Please cite this article in press as: T. Grüter, et al., Visual mental imagery in congenital prosopagnosia, Neurosci. Lett. (2009), doi:10.1016/j.neulet.2009.02.021 ARTICLE IN PRESS G Model NSL-25839; No. of Pages 6 Neuroscience Letters xxx (2009) xxx–xxx Contents lists available at ScienceDirect Neuroscience Letters journal homepage: www.elsevier.com/locate/neulet Visual mental imagery in congenital prosopagnosia Thomas Grüter a , Martina Grüter b , Vaughan Bell c , Claus-Christian Carbon a,∗ a University of Bamberg, Faculty of Humanities, Department of General Psychology and Methodology, 96047 Bamberg, Germany b Nottulner Landweg 33, D-48161 Münster, Germany c Department of Psychology (Box 078), Institute of Psychiatry, King’s College London, De Crespigny Park, London SE5 8AF, UK article info Article history: Received 13 October 2008 Received in revised form 5 February 2009 Accepted 6 February 2009 Keywords: Mental imagery Prosopagnosia Congenital Hereditary Agnosia Face recognition Expertise Dissociation Neuropsychology abstract Congenital prosopagnosia (cPA) is a selective impairment in the visual learning and recognition of faces without detectable brain damage or malformation. There is evidence that it can be inherited in an autoso- mal dominant mode of inheritance. We assessed the capacity for visual mental imagery in 53 people with cPA using an adapted Marks’ VVIQ (Vividness of Visual Imaging Questionnaire). The mean score of the prosopagnosic group showed the lowest mental imagery scores ever published for a non-brain damaged group. In a subsample of 12 people with cPA, we demonstrated that the cPA is a deficit of configural face processing. We suggest that the ‘VVIQ-PA’ (VVIQ-Prosopagnosia) questionnaire can help to confirm the diagnosis of cPA. Poor mental imagery, a configural face processing impairment and clinical prosopagnosia should be considered as symptoms of a yet poorly understood hereditary cerebral dysfunction. © 2009 Elsevier Ireland Ltd. All rights reserved. Prosopagnosia is a selective impairment in the visual learning and recognition of faces. It is associated with right or bilateral cerebral tissue damage to the temporal lobe (for an overview see [6,16]). McConachie [41] described the first case of prosopagnosia in a person without any detectable brain damage. She called this type of prosopagnosia “developmental”. By 2003, seven more single cases had been published [34]. As the term “developmental” was also used for acquired prosopagnosia in children, some authors pre- ferred the term “congenital” for cases without detectable brain damage [1]. There is now substantial evidence for a hereditary type of prosopagnosia [15,23]. All pedigrees published so far are com- patible with a simple autosomal dominant mode of inheritance, suggesting a single gene defect. A change in a single gene may indeed cause complex patterns of agnosias and/or apraxias. For example, a point mutation in the FOXP2 gene causes a complex disorder of speech production and language understanding [18,35]. Congenital prosopagnosia is not a rare disorder, although it was overlooked for a long time [24]. The prevalence of the condition in Germany was determined to be about 2.5% [30]. In an initial study we presented 38 people with congenital prosopagnosia of a familial type (hereditary prosopagnosia) iden- ∗ Corresponding author at: Faculty of Humanities, Department of General Psy- chology and Methodology, Markusplatz 3, 96047 Bamberg, Germany. Tel.: +49 951 863 1860. E-mail address: ccc@experimental-psychology.com (C.-C. Carbon). tified by a typical pattern of clinical symptoms [23]. Eight of them were tested with a battery of face recognition tests revealing an objective face recognition impairment in each one of them. One finding of particular interest has been the strikingly lower vivid- ness of visual mental imagery (VVMI) which was assessed with a modified VVIQ (Vividness of Visual Imagery Questionnaire [40]). The pattern of the impairment was somewhat inconsistent, though. While all prosopagnosic participants showed a VVIQ score of at least 1.5 SDs below controls (seven even > 2 SDs) for faces, three reported a normal VVIQ for non-face items. The effect did not seem to be familial, because one of the monozygotic twins in the study reported a normal imagery for non-face objects, the other scored 2 SDs below the controls’ mean. Visual mental imagery is a complex brain function involving sev- eral associative visual brain areas including the secondary visual cortex [32,45] and, as some have suggested, the primary visual cor- tex as well [31,33]. It is a distributed, modular system sharing some, but not all functional units with visual perception [27]. Brain dam- age can cause a total or partial loss of function [22], sometimes leading to dissociations in mental imagery abilities (cf. [28]). Levine et al. [39] reported on two patients with a dissociation of mental imagery after cerebral damage. One suffered from prosopagnosia and loss of mental imagery for faces and objects, while orientation in space, mental rotation and mental navigation was unaffected. The second showed the reverse pattern of impairments. Barton and Cherkasova [2] studied the accuracy of mental imagery in 9 people with acquired prosopagnosia. One participant 0304-3940/$ – see front matter © 2009 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.neulet.2009.02.021