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Cognitive Neuropsychiatry
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The role of affect and reasoning in a patient with a delusion of misidentification

Nora Breen , Diana Caine & Max Coltheart Published online: 09 Sep 2010. To cite this article: Nora Breen , Diana Caine & Max Coltheart (2002): The role of affect and reasoning in a patient with a delusion of misidentification, Cognitive Neuropsychiatry, 7:2, 113-137 To link to this article: http://dx.doi.org/10.1080/13546800143000203

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COGNITIVE NEUROPSYCHIATRY, 2002, 7 (2), 113137

The role of affect and reasoning in a patient with a delusion of misidentification

Nora Breen
Macquarie University and Royal Prince Alfred Hospital, NSW, Australia

Diana Caine
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Royal Prince Alfred Hospital and University of Sydney, Australia

Max Coltheart
Macquarie University, NSW, Australia
Introduction . This study investigated a patient with a delusion of misidentification (DM) resembling a Capgras delusion. Instead of the typical Capgras delusionthe false belief that someone has been replaced by an almost identical impostor patient MF misidentified his wife as his former business partner. Method. Detailed investigation of MFs face processing, affective response and affect perception, and ability to evaluate, and reject, implausible ideas was undertaken. Results. MFs visual processing of identity, gender, and age of familiar and unknown faces was intact but he was unable to identify the facial expressions of anger, disgust, and fear, or to match faces across expressions. MF also showed a reduced affective responsiveness to his environment, and impaired reasoning ability. Conclusions . We propose that MFs delusion of misidentification resulted from a combination of affective deficits, including impairment of both affective response and affect perception, in addition to an inability to evaluate, and reject, implausible ideas. These deficits, in combination with specific life events at the time of onset of the delusion, may have contributed to the form and content of the delusion. In addition, the results raise the possibility that the processing of face identity and facial expression are not as independent as previously proposed in models of face processing.

Correspondenc e should be addressed to Nora Breen, Macquarie Centre for Cognitive Science (MACCS), Division of Linguistics and Psychology, Macquarie University, Sydney, NSW 2109, Australia. Email: nora@maccs.mq.edu.a u # 2002 Psychology Press Ltd http://www.tandf.co.uk/journals/pp/13546805.html DOI:10.1080/13546800143000203

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Delusions of misidentification (DM) are a group of fascinating disorders in which there is a mistaken belief in the identity of oneself, other people, places, or objects. Of all the different forms of DM, the Capgras delusionthe false belief that someone, often a close relative, has been replaced by an almost identical-looking impostorhas received both the most attention and the most rigorous scientific investigation. Much of this work has attempted to delineate the factors underlying the form and content of these delusions, with a particular focus on face perception. The innovative testing of face processing in these patients has further enabled a more sophisticated understanding of normal face recognition (Breen, Caine, & Coltheart, 2000a; Ellis & Young, 1990). Recent work on the Capgras delusion has revolved around Ellis and Youngs (1990) proposal that the Capgras delusion might arise from a loss of the normal affective (autonomic) response to familiar faces. In these circumstances, the patient would have the conflicting experience of recognising a known face (such as that of their spouse), but without any accompanying affective response, leading them to conclude that the person was an impostor or double. In confirmation of this hypothesis, two independent research groups (Ellis, Young, Quayle, & de Pauw, 1997; Hirstein & Ramachandran, 1997) have now documented reduced skin conductance response (SCR) to known faces in patients with the Capgras delusion. This absent affective accompaniment to seeing a known face was described by Stone and Young (1997) as constituting anomalous perceptual experiences created by a deficit to the persons perceptual system. (p. 327). Although the concept of a perceptual anomaly successfully captured a salient aspect of the patients experience, it also elided two potentially separable sources of distorted or impoverished information: externally derived incoming sensory information (visual, auditory, tactile), more usually described as perceptual, and internally derived autonomic information with its cognitive correlates, more usually described as affect or emotion. We have previously reported two cases of mirrored-self misidentification (Breen et al., 2000a, 2001), a delusion involving the false belief that your own reflection is another real person. In these studies we clearly distinguished between the terms perceptual and affective in order to explore the possible contribution to the DM from both of these sources. Both cases (FE and TH) had perceptual abnormalities that, we argued, to some extent determined the form and content of the delusion, although the actual abnormality varied dramatically between the two casesFE had a dramatic impairment in the perceptual processing of faces, whereas TH had an inability to interpret reflected space, a mirror agnosia. These two cases thus also demonstrated that very different perceptual abnormalities could give rise to very similar delusional phenomena. In addition both FE and TH tended to judge unknown faces to be personally familiar. As we have discussed in detail elsewhere (Breen et al., 2001), a sense of familiarity occurs when viewing a personally known face as a

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result of activation of the corresponding face recognition unit (FRU) (Bruce & Young, 1986), indicating that the face has been previously seen, and from generation of the affective response corresponding to that particular person. Whereas FEs false recognition may have resulted directly from his impaired perceptual processing of facesdegradation in the structural encoding of faces may have led to unfamiliar faces causing erroneous activation of the FRUs of known peopleTHs structural encoding of faces was entirely intact. We suggested that THs false recognition of unfamiliar faces resulted from an inappropriately modulated affective response to all faces, and therefore an erroneous sense of familiarity in response to a strangers face. Whereas Ellis and Young have focused on the loss of the affective response, our work with TH suggested that an excess of affective responsiveness equally might underlie the formation of a misidentification delusion. When considering abnormal affective experience in patients with DM it is important to consider two dissociable factorsones emotional response to the environment and the ability to recognise emotional (face) expressions in others. To distinguish between them, we propose to use the terms affect perception and affective response. Using this terminology, affect perception refers to the subjects ability to read emotional expressions on the faces of others. On the other hand, the affective response refers to the subjects emotional responsiveness to the environment. Although it has been argued that the affective response to known faces can be relatively selectively impaired (Ellis et al., 1997), it is likely that patients with brain damage causing a global flattening of responsiveness towards the environment would concomitantly have a decreased autonomic response to well-known faces. Support for this contention comes from work with patients with bilateral ventromedial frontal lobe lesions demonstrating that in addition to the more specific deficit in skin conductance response to familiar faces (Tranel, Damasio, & Damasio, 1995), these patients also fail to produce the normal skin conductance response to emotionally charged visual stimuli, such as pictures of mutilation and social disaster (Damasio, Tranel, & Damasio, 1991). However, if altered affective response is implicated in the formation of DM, this finding raises the question of why the patients with ventromedial damage are not delusional. One possibility is that the loss of affective response to familiar faces is not crucial for the formation of the Capgras delusion. This seems unlikely in that, although to date only six Capgras patients have been tested (Ellis et al., 1997; Hirstein & Ramachandran, 1997), the finding of reduced SCR to familiar faces has been surprisingly consistent. Alternatively, patients with ventromedial lesions may lack additional contributing factors necessary for the formation and maintenance of a delusion, factors that have not yet been fully delineated. One contributing factor to the phenomenology of a DM, in addition to perceptual and affective abnormalities already described, is likely to be some form of defective reasoning, although this idea has yet to be satisfactorily

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explored or explained (Breen et al., 2000b; Langdon & Coltheart, 2000; Young, 1998). Young et al. (1993) have argued that the failure of reasoning is generated and maintained by a particular mood, but the question of whether reasoning as such in these patients may be impaired has never been addressed. For instance, although it is easy to imagine that an abnormal experience initially leads to a false belief, it is difficult to imagine that one would not rapidly evaluate the belief as false and subsequently reject it. There are many reports in the literature of non-delusional patients with perceptual and/or affective deficits that are very similar, if not identical, to those described in patients with DM. It would appear that patients with DM lack insight regarding their perceptual and/or affective deficits that might have helped them to override their false belief(s) arising from their abnormal experience. For example, patients with prosopagnosi a are unable to recognise their own face in a mirror yet generally are not reported as having the delusion of mirrored-self misidentification, presumably because they are aware that a neurological condition is preventing them from correct recognition of faces. In contrast, it would appear that patients with DM are unable to reject a belief on the grounds of its implausibility and inconsistency with everything else that they know. This is highlighted by reports of patients with DM who appreciate that others find their belief bizarre, yet strongly adhere to their delusion and cannot be persuaded that their belief is false (Alexander, Stuss, & Benson, 1979; Young, 1998). In our earlier work with patients with DM (Breen et al., 2000b, 2001), we investigated the question of perceptual and affective abnormalities with respect to face processing underlying the delusion of mirrored-self misidentification. In the present study, we sought to investigate further the role of impaired processing of affect, both in terms of affective response and affect perception, and the nature of the inability to reject a belief once established, in the production of a DM. The case study presented had a variant of the Capgras delusion that had remained stable for 10 months prior to our investigations.

CASE STUDY Background

At the time of testing, MF was a 68-year-old man, who had been married for 42 years, and had three adult children. He was a practising litigation attorney at the time he suffered his head injury (four years prior to the current assessment) and had up to that time enjoyed an extremely successful and high-profile career, which included an 8-year partnership in a law firm with JY, a female attorney. His only significant medical history prior to the head injury was a short episode of left-sided weakness in 1996 that his general practitioner attributed to a small stroke. In December 1997, MF sustained a severe head injury when he fell an estimated 20 feet from a ladder, landing on a concrete

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driveway. On arrival at hospital his Glascow Coma Score (GCS) was initially 11, but then deteriorated. An initial CT brain scan showed bifrontal and right temporal lobe contusions, and MF was immediately ventilated, intubated, and put into an induced coma. A repeat CT brain scan the following day showed massive bifrontal contusions, and posterior displacement of the ventricles. A left frontal lobectomy was performed to alleviate intracranial pressure due to swelling and haemorrhaging. A further left frontal lobectomy was performed three days after his admission due to continued mass effects from swelling. He remained comatose and on ventilation for 2 months. He had a number of medical problems during that time including hydrocephalus (and subsequent ventricular-peritoneal shunt insertion), atrial fibrillation, intermittent elevations in intracranial pressure, shunt infections, pancreatitis, and staphylococcal infection. He had a feeding gastrostomy inserted due to swallowing difficulties and had a urinary catheter for 18 months. He suffered bilateral hearing loss secondary to the head injury, and now wears bilateral hearing aids. He had several neurosurgical operations over the next 2 years including insertion of a metal grate implanted over the left frontal surgical site, and subsequent debridement of the infected skull plate. He suffered several post head injury seizures. A CT brain scan 2 years post injury reported chronic encephalomalacic changes in the frontal lobes bilaterally, a small calcified subdural haematoma in the left frontal region, mild cerebellar atrophy, and a lacunar infarct within the pons (see Figure 1). MF remained in an acute care hospital for 2 months, followed by 8 months in a rehabilitation hospital, and then nursing home care. A neuropsychologica l assessment 6 months post head injury demonstrated that he was oriented, had a mildmoderately impaired attention span, a mild memory impairment, and severely impaired constructional skills. With regard to language, his naming was entirely normal, but he showed mildly impaired comprehension and impaired repetition. His ability to make common sense judgements was normal but he had a mild reasoning deficit. He was discharged home 1 year post injury. He was initially wheelchair-bound, and received physiotherapy, speech therapy, and nursing care. He made a dramatic recovery at home, and 4 years after the operation had achieved independent mobility and was independent in activities of daily living. Following his head injury, MF showed a number of behavioural changes consistent with bilateral frontal lobe damage. He had a flattened affect, although he retained an appropriate, and often witty, sense of humour. His family described his emotional responsiveness post head injury as blunted, and said that his emotional warmth and interaction with them was somewhat reduced. He lacked initiative but was cooperative with activities that were arranged for him. He occasionally made insensitive comments and appeared unaware that he had hurt peoples feelings, even when he had reduced someone to tears after an insulting remark.

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Figure 1. CT brain scans for patient MF, 9 months post head injury. Scans show severe trauma to the frontal lobes bilaterally, with the left side affected to a greater degree than the right. Lacunar infarcts are present within the pons on the right side of the midline and also in the right thalamus. The ventricular-peritonea l shunt is present within the body of the right lateral ventricle.

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The delusion
In June 1999, 18 months post head injury, MF underwent an operation to remove his gastrostomy feeding tube, an operation that involved a general anaesthetic. When MF woke from the anaesthetic after the operation his wife was sitting next to his bed. He exchanged some pleasantries with her and then asked her if she had seen his wife! From that point on, and for the next 10 months, MF believed that his wife was JY, his former partner in the law firm. This was the only misidentification that MF displayed. He correctly identified all of his other relatives including his three children, his grandchildren, his mother-in-law, and sister-in-law, and had no difficulty identifying friends and acquaintances. Interestingly, his wifes name was Joan and his former business partners name was Joanne. His wife is 13 years older than JY, but the two women are of similar colouring and build. His wife has reddish-blonde, short, wavy hair whereas JYs red hair was longer and straight. MF and his wife have three children, as do JY and her husband. MF said that although he respected JYs ability as an attorney, he intensely disliked her on a personal level. In the eight years that MF and JY were law partners, they had only one social meal together. MF did not protest about leaving the hospital with the woman he thought was his former business partner and was reasonably happy for her to look after him as long as she was not too affectionate towards him. Not long after the gastrostomy operation, he and his wife were in the small elevator in their multistorey home. His wife was unclear at this stage whether MFs delusion was constant or intermittent and, as she felt they were getting along very well at that moment, she leaned towards him, put her hand on his arm, and attempted to kiss him. MF reacted angrily, backed away from her waving his cane in a menacing way, and threatened to strike her. The following day, MF told his doctor about the incident stating that she was all over me. When the doctor light-heartedly responded that most men would be thrilled if their wives wanted to kiss them, MF replied, I would be happy too if it was my wife, but it wasnt my wife, it was JY. If my wife knew about this she would not have appreciated it! His wife was forced to move out of their bedroom as MF refused to sleep in the same room with her. MFs wife and children constantly tried to reason with him but were unable to convince him that the woman living with him was his wife and not JY. Ownership of their home was transferred to his wifes name at this time, and MF was angry for several weeks, as he believed that JY now had control of the house that he and his wife owned. Occasionally, MF asked his wife (whom he thought was JY) where his wife was, but he never made an attempt to find her. When he was asked where his wife was, he said that she was in the other house, a house that he described as having exactly the same address, including street number, street, and suburb, as the house in which he currently lived. He described the two houses as identical except that the house he currently lived in had two storeys, whereas the house

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his wife lived in had three storeys. He identified all of the mens clothing in his bedroom as being his own and said that all of the womens clothing was JYs and did not belong to his wife. He said that all of his wifes clothing and her belongings were at the other house. When MF was told by the examiner that she found his storythat JY had taken him home from hospital and taken care of him since the head injury, and that his wife now lived in another house that had the same address as the one he currently lived inbizarre and extremely hard to believe, MF agreed that it was strange, but nevertheless insisted that it was true. MFs indifference to constant questioning about his delusion was striking. He was unperturbed when evidence contradicting his delusion was pointed out to him, and when the examiner repeatedly emphasised the improbability of the delusion. MF understood that the research the examiner was conducting involved investigating delusions, and he understood that the particular delusion being investigated was his belief that the woman living with him was not his wife. However, he repeatedly told the researchers that he did not have a delusion, and that they should investigate the real delusionthe delusion held by his former business partner, JY, who believed that she was his wife!

Neuropsychological testing
Neuropsychological testing was undertaken to evaluate MFs performance in a range of cognitive domains (see Table 1). Based on his education and employment history, MF was estimated to have a high averagesuperior intellect prior to his head injury. Testing revealed that his current intellect was in the average range, as assessed by measures of verbal (NART) and non-verbal (Ravens Coloured Progressive Matrices; Raven, 1947) ability. Attention and Intellectual Function. MFs verbal attention span was limited and below the average range, but his visual attention span was in the average range (WAIS-R; Wechsler, 1981). His manual speed was very slow as demonstrated on a timed copying test (WAIS-R Digit-Symbol subtest). MF performed in the average range on tests of language. In contrast, his performance on a test of mental arithmetic was very impaired, and well below the expected average range. MFs basic visuo-perceptual skills were intact as evidenced by his intact clock drawing and copying of various pictures (including a bicycle and 3-dimensional cube) and he was able to identify missing components in line drawings (Picture Completion, WAIS-R). His constructional skills were somewhat less robust (Block Design, WAIS-R). Memory . MF demonstrated a dissociation between his verbal and nonverbal memory function. His verbal memory for stories and word associations was intact in the high average to superior range (WMS-R; Wechsler, 1987), as

A DELUSION OF MISIDENTIFICATION TABLE 1 Results of tests of cognitive ability for patient MF

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Tests IQ Language NART (Estimated Full Scale IQ) Test of Reception of Grammar (TROG) WAIS-R Vocabulary Mental Arithmetic Similarities

Patient MF 107 (Average) 73/80 (91%) MOANS ASS 8 4 9 75 %tile 10/10 intact intact 29.5* MOANS ASS 13 10 8 4 MOANS ASS 5 (5 forward, 3 backward) 9 (7 forward, 6 backward) MOANS ASS 14 14 12 13 12 9 (12) 8.5* Personal Sem 15 borderline 17 Acceptable 21 Acceptable 53 Acceptable Raw Score 49 41 Autobiograp h 7 Acceptable 9 Acceptable 6 Acceptable 22 Acceptable ASS 15 9 (Continued overleaf)

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Non-verbal skills

Ravens Coloured Progressive Matrices Clock drawing Copying of shapes, designs and bicycle Copy of 3-dimensiona l cube REY Complex Figure TestCopy WAIS-R Picture Completion Picture Arrangement Block Design Digit Symbol

Attention/ Concentration Memory

WMS-R Digit Span Visual Span WMS-R Logical Memory I Logical Memory II Verbal Paired Associates I Verbal Paired Associates II Visual Reproduction I Visual Reproduction II (Visual Reproduction II, with prompt) Rey Complex Figuredelayed recall Autobiographica l Memory Interview Childhood Early Adult Life Recent Life Total Warrington Recognition Memory Test Words Faces

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Tests Executive Function Trails A (ASS) Trails B Controlled Oral Word Association Test Total in 3 minutes (phonemic cue) Animal FluencyTotal in 1 minute Colour Form Sorting Ttest 7 incomplete 13 9

Patient MF

ASS 3

1st percentile < 10th percentile

2/2 categories 0 96 1st percentile 94 < 1st percentile

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Wisconsin Card Sorting Test Number of categories completed Errors Perseverative Errors

The scores reported for MF on the Wechsler Adult Intelligence ScaleRevised (WAIS-R), Wechsler Memory ScaleRevised (WMS-R) are age-scaled scores (ASS) as reported in the Mayo Older American Normative Studies (Malec et al., 1992; Ivnik et al., 1992a, 1996). *within 1 sd of mean. (AMIPersonal Sem for Personal Semantic, AutobiographAutobiographical). The scores reported for MF on Trail Making Test (TMT) and Controlled Oral Word Association Test (COWAT) are age-scaled scores (ASS) as reported in the Mayo Older American Normative Studies (Malec et al., 1992; Ivnik et al., 1992a; Ivnik et al., 1992b; Ivnik et al., 1996). *within 1 sd of mean.

was his recognition memory for words (WRMT; Warrington, 1984). In contrast, he demonstrated a deterioration of newly acquired visual information over time. His immediate recall of the WMS-R line drawings was in the high average range, however his recall dropped to a low averageaverage level after a short delay. Similarly, his delayed recall of the Rey Complex Figure was very impoverished. In contrast to his very good recognition of words (ASS 15) on the WRMT, he only achieved an ASS 9 on recognition memory for faces. His autobiographical memory for childhood, early adult life, and recent life was entirely intact (AMI). His personal semantic memory was in the borderline range for his childhood, but was entirely intact regarding both his early adult and recent life. Executive function. MFs most striking deficits occurred on tests of executive function. He was accurate, although slow, on Trails A, but made many errors, became hopelessly confused, and eventually abandoned Trails B after almost 5 minutes. His phonemic and category fluency were very impoverished. He achieved the required two categories on the simple CFST, but did not achieve a single category on the Wisconsin Card Sorting Test, a performance

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that was dominated by perseverative errors. His performance on the Wisconsin Card Sorting Test is discussed in more detailed later in the section on Investigation of reasoning ability.

INVESTIGATION OF FACE PROCESSING

MFs face processing abilities were thoroughly investigated using a combination of standardised and experimental tests. MFs performance on these tests was compared to his wifes performance when appropriate (for example, recognition of personally familiar faces) or compared to a normative sample of five males, matched for age and educational attainment (see Appendix 1 for the normative sample demographics). The results of MFs face processing tests are presented in Table 2.

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Experiment 1: Face matching

Face matching was assessed with the Benton Facial Recognition Test (Benton, Hamsher, Varney, & Spreen, 1983), a difficult test where cues are limited (matching is conducted on the basis of facial features only, as hairstyles have been removed), the lighting is varied (often resulting in significant portions of the faces being covered by dark shadow), and the faces are often in very different orientations. Results. MF performed in the normal range on this test.

Experiment 2: Identification of age of unfamiliar faces

Ten black and white photographs of unfamiliar faces (with no other identifying cues) were presented in a random order and the subject was asked to give an approximate age for each of the faces. Results. The age approximations on this test are subjective. MFs age identifications for eight of the faces were within the range of ages reported by the five control subjects. His age approximations for the other two faces were very close to the age range provided by the controls (4 years and 9 years outside the range). As eight of MFs age approximations were inside the range provided by the control subjects, and as this measure is subjective and the control group small, we have concluded that MFs age identification of faces is within the normal range.

Experiment 3: Famous face identification

Identification of famous faces was assessed with an experimental test consisting of photographs of the faces of 22 famous people and 22 matched unknown

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BREEN, CAINE, COLTHEART TABLE 2 Results for the face-processing tests

MF Face Matching Age Identification Facial Recognition Test (Benton) (Unfamiliar faces) 47 Age (years) 1 1 18 12 35 25 35 45 70 70 12/12 12/12 19/22 17/22 56/59 (/10) 10 8 10 7 7*** 5** X Matching Faces Across Expressions Neutral Expression Same Expressions Different Expressions 33* 25*** 23***

Controls Range 4154 Range (years) 15 13 1320 38 2045 2535 4455 4560 5575 7084 MFs wife 12/12 12/12 19/22 18/22 56/59 Controls (n = 5) 9.8 (sd 0.45) 8.2 (sd 1.64) 9.2 (sd 1.09) 8.2 (sd 1.30) 9.3 (sd 0.55) 8.2 (sd 1.30) Controls (n = 5) sd 38.0 38.8 37.2 (2.45) (1.79) (3.35)

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Face Recognition

Personally familiar (family members) Identified as familiar Named Famous Identified as familiar Name/Specific semantic identification Unfamiliar (strangers faces) Identified as unfamiliar

Facial Affect Perception

Ekman & Friesen Facial Affect Photos Happy Sadness Surprise Anger Disgust Fear

*significant at p < .025, **significant at p < .01, ***significan t at p < .0001. The Calder et al. (1996) subset of Ekman and Friesen Pictures of Facial Affect was used.

people. The unknown faces were matched according to gender, age, and physical similarity. An additional 37 unknow n faces, not matched to the famous faces, were also included in the test, making a total of 59 strangers faces. Each photograph was modified to produce black and white pictures of the face with no other identifying cues (e.g., body parts, contextual cues). The faces were

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presented in a random order, one at a time, in the centre of a PC laptop screen. The examiner controlled the rate of the stimuli presentation and each face remained on the screen until the subject identified whether it was familiar or not. If the face was identified as familiar, the subject was asked to provide a name and/or identifying semantic information. MFs wife acted as the control subject on this test. Results. MF identified 19/22 of the famous faces as familiar and provided the correct name or identifying semantic information for 17/22, a performance no different from that of his wife. MF correctly identified 35/37 strangers faces as unfamiliar, which was also consistent with his wifes performance on this test. MF and his wife each incorrectly identified two strangers faces as familiar, saying that the faces looked familiar but they did not know who they were.

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Experiment 4a: Identification of personally known faces

MFs ability to identify personally familiar faces was examined with an individually tailored test. Photographs of his immediate family members, relatives, close friends, and JY, were modified to produce black and white photographs of faces without any other cues (e.g., body parts, contextual cues), and 12 appropriate photographs were thus obtained. Each known face was matched with an unfamiliar face of the same sex, approximate age, and physical likeness. An additional 12 unknown faces were also included in the test, making a total of 22 strangers faces. The faces were presented one at a time, in a random order, on a PC laptop screen. The subject was initially asked whether each face was familiar or not, and then to provide as much information as possible, including the name, about the individuals whose faces they classed as familiar. This test was administered to MFs wife who acted as the control subject. Results. MF correctly identified all 12/12 personally familiar faces as familiar and provided the correct name for each, including his wife and JY. His wife was also 100% correct on this test. MF correctly identified 21/22 strangers faces as unfamiliar, a performance no different from that of his wife.

Experiment 4b: Identification of persons implicated in the DM from photographs

MF was further tested on his ability to identify his wife and JY in photographs. Colour photographs, which had been taken by MFs family members, were presented in which his wife and JY appeared either individually in different photographs or together in the same photograph. When MF was shown a

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photograph that included MF, his wife, and JY, he was asked to identify which person in the photograph was sitting beside him during the testing (his wife). Results. MF correctly identified his wife and JY in several photographs in which they appeared individually. He made these identifications rapidly and confidently. When shown a photograph of himself, his wife and JY together, he correctly named each person. When he was asked to point to the person in the photograph who was the same person as the one sitting next to him during the testing (his wife), he pointed to JY. The examiner asked MF several times whether he was sure that the person he had indicated in the photograph (JY) looked exactly the same as the woman beside him (his wife), and MF remained adamant that they were the same person. The examiner pointed out to MF that, in her opinion, the photograph of JY did not look like the woman sitting next to him, but rather the photograph of his wife looked identical to the woman who was sitting next to him. MF said that he did not agree. His reaction was unperturbed, and he did not get agitated when confronted in this way.

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Experiment 5: Identification of facial expressions (affect perception)

MFs ability to identify facial expressions was tested with Ekman and Friesens (1976) black and white photographs of unfamiliar people expressing emotion. We used the Calder et al. (1996) subset of 60 of the Ekman and Friesen faces, which included pictures of 10 models faces (5 women, 5 men). For each face, there were poses corresponding to each of six emotions (happiness, sadness, disgust, fear, anger, and surprise). The names of the six emotions were printed on a card and this was placed in front of the subject throughout the test. The subject was shown each of the photographs one at a time, in a random order, and asked to decide which of the six emotion names best described the facial expression shown. There were 60 trials (one for each of the six emotions for each of the 10 models), leading to an accuracy score out of a possible maximum of 10 for each of the six emotions. Results. MFs ability to identify the facial expressions happiness, sadness, and surprise did not differ from the controls. In contrast, MFs ability to identify anger was only borderline and he was significantly worse than controls in his ability to identify the facial expressions disgust and fear (see Figure 2).

Experiment 6: MFs understanding of the emotions he was impaired at identifying

MFs difficulty in recognising some facial expressions did not appear to reflect failure to comprehend emotional terms: he gave examples of occasions when he would feel angry, disgusted, and afraid, and could describe circumstances in which other people would experience the same emotions.

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Figure 2.

Face expression recognition.

Experiment 7: Matching faces across expressions

This test consisted of pairs of faces (black and white photographs from the Ekman and Friesen series), presented side by side, so that for each trial two face images were presented simultaneously until MF made a response. This test had three different conditions: 1. Neutral Expression; two pictures either of the same person or of two different people (same gender), with the same neutral expression. 2. Same Expression; two pictures either of the same person or two different people (same gender) with the same facial expression (happy, sad, disgust, fear, anger, or surprise). 3. Different Expression; two pictures either of the same person or two different people (same gender) each having a different expression (happy, sad, disgust, fear, anger, or surprise). In each condition the subject was asked to decide if the two faces had the same identity (that is, whether or not they were pictures of the same person) regardless of the facial expression. The items were presented in a random order, and the subject gave a verbal response. Results. In contrast to his good performance on other tests of face recognition, MF demonstrated a significant interference effect of facial expression when making judgements about face identity. When the two faces were expressionless (neutral expressions) he was 83% accurate in discriminating whether the face identity was the same or different. In striking contrast, he was almost at chance (60%) at discriminating facial identity when the faces had an expression (see Figure 3). The interference effect was cumulative: he was 83%

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Figure 3.

Face constancy: Matching faces across expressions.

correct when both faces had a neutral expression; 63% correct when the two faces had the same expression; and only 58% (almost chance) if the two faces had different expressions. As MF performed in the normal range on the more difficult Benton Face Recognition Test (indicating intact face matching on the basis of limited cues, varied lighting, and different orientation), it was somewhat surprising that his ability to match faces in the Neutral Expression condition of this test was not equal to that of the controls. It should be noted that had MF achieved one more correct match on this Neutral Expression subtest of the Matching Faces Across Expressions test, his performance would not have been significantly different from that of the controls. Nevertheless, his below average performance on this subtest remains puzzling. The only difference between the two tests was in the administration, in that the easier neutral Matching Faces Across Expressions test was intermixed with matching of faces with various expressions. As we will discuss later, MF was impaired at identifying facial expressions and very impaired at discriminating between faces when the face identities were discrepant with the facial expressions (e.g., same identities, different expressions). It may be that the presentation of the neutral expression face stimuli among faces with expressions in the experimental Matching Faces Across Expressions test interfered with MFs ability to perform the neutral facematching test to the best of his ability.

INVESTIGATION OF REASONING ABILITY

MF demonstrated average level verbal reasoning and averagehigh average level reasoning about visually presented material on standardised subtests of the WAIS-R. This included tests of visual reasoning (Picture Completion, ASS 13), logical sequencing of events, which included anticipating the consequence of

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actions and distinguishing essential from irrelevant details (Picture Arrangement, ASS 10), and conceptual reasoning (ASS 9).

Experiment 8: Affect-neutral reasoning test

The Wisconsin Card Sorting Test was administered to assess MFs reasoning ability on an affect-neutral task. Results. MF was grossly impaired on this test. He perseverated to an incorrect category for the entire test, 128 consecutive trials. His performance indicated impaired error monitoring and set shifting, and inability to adapt his own performance according to external feedback.

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Experiment 9: Plausible/implausible reasoning test

An experimental test of reasoning ability was devised to assess MFs ability to evaluate information (provided in story format) that could be interpreted in two ways, to make either a plausible or implausible conclusion. One of the stories was specifically tailored to MFs delusion in order to investigate whether his delusional belief (that his wife had been replaced by someone else) was a circumscribed belief, or whether the belief was more generalised (i.e., whether he believed that other people could have relatives replaced by impostors). The four stories that made up this test are presented in Appendix 2. The subjects had unlimited time to read the story and select their response (by circling). Results. The results are presented in Table 3. All five of the controls chose the plausible conclusion for Story 1, and 4/5 chose the plausible conclusion for both Story 3 and Story 4. One control subject for both Story 3 and Story 4 chose the cant tell option, indicating that he did not feel he had enough information to reach a conclusion. It is important to note that none of the control subjects chose the implausible conclusion for any of the four stories. In contrast, MF selected the cant tell option for Stories 1, 2, and 4. In addition, he chose the implausible conclusion for the scenario that was tailored towards his own
TABLE 3 MFs performance on the experimental plausible/implausible reasoning test

MF Subject 1 Story Story Story Story 1 2 3 4 Cant tell Cant tell No Cant tell No No Yes No Subject 2 No No Yes No

Controls (n = 5) Subject 3 No No Yes Cant tell Subject 4 No No Cant tell No Subject 5 No No Yes No

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delusional belief, indicating that he believed that identical-looking impostors could replace other peoples wives.

DISCUSSION
Following the work of Ellis and Young (1990) on face processing deficits in Capgras patients, and our own positive findings in two cases of mirrored-self misidentification, we examined in detail MFs ability to discriminate and recognise faces. MF was able to identify both personally familiar and famous faces, to discriminate between familiar and unfamiliar faces, and to identify the approximate age of unfamiliar faces. Although his ability to match unfamiliar faces was inconsistent, he was able to match unfamiliar faces with neutral expressions on the most difficult face matching test (Facial Recognition Test, Benton 1983). As visual face processing per se seemed to be intact, we proceeded to investigate the possible contribution of altered affect in MF. We did this in three ways. We interviewed his family about his affective responsiveness towards them; we interviewed MF with respect to his understanding of emotion; and we looked at his ability to recognise facial affect (affect perception). Although he appeared to understand the difference between emotions, in that he was able appropriately to describe occasions when he or others would feel different emotions, his family reported that there was generalised blunting of emotions, with little emotional expression, dulled responses in emotional situations, and reduced emotional warmth towards his children and other close relatives. As previously discussed, it is likely that MFs global dampening of affective responsiveness to the environment encompasses a reduction in affective response (SCR) to familiar faces, as has been documented in other patients with ventromedial frontal lobe damage (Tranel et al., 1995). To that extent he can be thought of as being like more typical Capgras patients. It is further possible that MFs delusional belief that he was not living in his own home was directly related to this reduced responsiveness to the environment. MF may have resolved the discrepancy of living in a house that looked like his own house with the same address as his house, but that didnt feel like his home, by generating the belief that he was living in a house that was somehow like his real home, but that his real home (where his wife lived) was somewhere else. In addition to reduced affective responsiveness, MFs ability to identify facial expressions was selectively impaired: while his identification of the facial expressions of happiness, sadness, and surprise were normal, his ability to recognise anger was only borderline, and he was frankly impaired at discriminating disgust and fear. His difficulty in recognising facial expression was not simply an exaggeration of the normal pattern: while control subjects in this study, and in studies by Calder et al. (1996) and Ekman and Friesen (1976), found anger and fear relatively more difficult to identify, they were easily able

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to identify the expression of disgust, which was second only to happiness (see Appendix 3 for control data for Calder et al., 1996, Ekman & Friesen, 1976, and our controls for this study). We investigated whether MFs difficulty in discriminating among some facial expressions might affect his otherwise intact face matching ability. This was indeed the case: MFs impaired expression analysis interfered with his ability to match faces and the interference effect was incremental. MF was best at matching faces with neutral expressions, more impaired if both faces had the same expression, and most impaired if the two faces had different expressions. Thus, MFs deficit in interpreting facial expression led him to mistake differences in expression for differences in identity, notwithstanding that he was able to make appropriate allowances for the effects of changes in orientation and lighting when matching faces with neutral expressions. We speculate that MFs impaired identification of some facial expressions, and his very impaired ability to discriminate face identity when the face showed an expression, are likely to be contributing to his misidentification of his wife as his former business partner. Interestingly, MFs wife provided some support for this when she was given feedback about his face processing deficits. MF had told his wife that she could not be his wife because his wife smiled a great deal whereas she never smiled. MFs wife reported that the observation was true in that since his head injury she was not the happy person she used to be: she had been forced to take on the role of head of the household, which included controlling their finances and making all the decisions. This was a role that she had never previously assumed or desired during their 42 years of marriage, and having to do so now caused her a great deal of stress and worry. However, other patients have been reported with either widespread problems in the recognition of facial expressions (Etcoff, 1984) or more specific deficits in the recognition of fear (Adolphs, Tranel, Damasio, & Damasio, 1994, 1995; Broks et al., 1998; Calder et al., 1996), disgust (Gray et al., 1997; Sprengelmeyer et al., 1997), or both (Sprengelmeyer et al., 1996, 1997), and are not delusional, although matching of identity across expressions has only rarely been tested. One non-delusional patient, DR, has been reported to have both a specific deficit in the recognition of fear and an impaired ability to match faces across identities, a profile very similar to that of MF (Young, Hellawell, van de Wal, & Johnson, 1996). The degree of deficit was different, however. While both MF and DR had significant deficits in the perception of facial expressions, DR was only mildly impaired in her ability to match face identities across different expressions, whereas MF was severely impaired on this task. Why did MF believe his wife was JY, and not a stranger who looked similar to his wife, or some other person that he knew? In addition to the physical similarity between the two women, and their similar names (Joan and Joanne), the nature of MFs attachment to JY may have contributed to her role in his delusional belief. MF and his family described him as having a strong, albeit

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negatively charged, emotional relationship with JY. During the 8 years that MF and JY ran their law firm, MF reported that he and JY had many confrontations due to personality clashes and conflicting ethical values. Although MF respected JY on a professional level, he said that he disliked her intensely on a personal level. In the same way that Capgras patients typically misidentify the person to whom they have the strongest positive or negative emotional attachment (Young, 1998), it is probably significant that MF misidentified his wife, to whom he had a very close positive emotional attachment, as a woman with whom he had an intensely negative emotional relationship. Such similarities between actual and delusional characteristics have been reported previously. Burgess, Baxter, Rose, and Alderman (1996) reported a man (PD) who, following a severe head injury, had the delusional belief that a fellow patient (Jake) was a male nurse (Jamie) who had previously cared for him (in a different institution), and that he was having an affair with PDs wife. Further, the wife of one of the nurses who had cared for PD had the same name as PDs own wife (Jane). It is further conceivable that the content of MFs delusion, and possibly other delusions of misidentification, may depend on specific life events occurring at the time of the onset of the delusion. Although prior to the head injury MF had not had contact with JY for 8 years, she regularly visited him following the head injury and while he was in PTA. She spent a substantial amount of time with him showing him photographs and mementos to assist his recall. MF does not have much conscious recollection of her visits during this time. He may have stored interlinked memories of his wife and JY during his period of PTA, memories that were most likely patchy and somewhat confused. Following the head injury and his recovery from PTA, MF was not delusional, and he correctly identified both his wife and JY. It was only 18 months after the head injury that the delusion arose, immediately following a surgical procedure that involved a general anaesthetic. The mild disorientation and confusion due to the effects of the general anaesthesia may have caused displacement of patchy recall of episodes from the period of PTA, and hence contributed to the misidentification of his wife as JY. A number of other cases have also been reported to have developed delusions while either still in, or immediately following, a period of PTA following a head injury (Box, Laing, & Kopelman, 1999; Burgess et al., 1996). We have argued here that perceptual and/or affective deficits, as well, possibly, as specific life events occurring at the time of onset of the delusion, can contribute to the form and content of the DM, yet they cannot be the entire explanation, as all of these occur in patients who do not develop delusional states. Patients with a DM must also have an additional deficit in reasoning that prevents them from evaluating, and rejecting, implausible beliefs. In this study, we had the opportunity to examine the reasoning of our patient with a DM on an affect-neutral taskthe Wisconsin Card Sorting Test. On this task DM

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perseverated to the incorrect category he first generated for the entire test (128 consecutive trials) despite feedback after every trial that his response was incorrect. His reasoning was defective precisely in the sense that once he had formed a belief (in the case of the Wisconsin Card Sorting Test, that a particular category was the required one), he could not subsequently evaluate or change it. This behaviour also characterised his DM: he clung tenaciously to the DM for 10 months, and no amount of evidence to the contrary could persuade him otherwise during that time. MFs reasoning was further tested with the experimental reasoning test. For the story that was closest in content to his own delusional belief MF chose the implausible conclusion; that is, he believed that an impostor had replaced the mans wife in the vignette. MFs (implausible) response for this story was in contrast to his cant tell response for the three other stories, suggesting that MF may have identified with the fictitious man in Story 2, and responded to the story scenario in the same way that he reacted to his own situation. For the three stories that were not directly related to his own delusional belief, MF, in contrast to controls, was not able to discard the implausible aspects of the story to come to the more plausible conclusion. These data together indicate a more pervasive reasoning deficit than has previously been suggested (see for example Young et al., 1993; Stone & Young, 1997). As demonstrated by Ellis and Young (1990) and by Breen et al. (2000a), the study of DM has both profited from and made a contribution to models of normal face processing. Until recently, the dominant cognitive model of face processing (Bruce & Young, 1986) only incorporated a role for affect in relation to facial expression analysis. It proposed that expression analysis was independent from, and not important to, either the recognition of familiar faces or the processing of unfamiliar faces, both of which utilised separate, and independent, cognitive pathways (Young et al., 1993). More recently, following the work of Ellis and Young, and based on the findings of a double dissociation between prosopagnosi c patients who demonstrated no overt face recognition yet intact autonomic responses (SCR) to familiar faces, and Capgras patients, who demonstrated intact overt face recognition but reduced or absent autonomic responses to familiar faces, we proposed a modification to the Bruce and Young (1986) model of face processing that included an intrinsic role for the processing of affect in face recognition. Although we have not yet tested whether MFs impaired facial expression analysis also interferes with his ability to identify famous or personally familiar faces, a task he has no difficulty with when the faces had neutral expressions, the data on MF raise further questions about the possible role of affect perception in face processing. The finding that although he could efficiently discriminate unfamiliar faces with neutral expressions, he was unable to match faces showing expressions, suggests that facial expression analysis may not be as independent of other aspects of face processing as previously thought.

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CONCLUSION
In conclusion, we have proposed that MF has a delusion of misidentification resulting from a combination of affective deficits, including impairment of both affective response and affect perception, in addition to an inability to evaluate, and reject, implausible ideas. The nature of his underlying deficits, in combination with specific life events at the time of onset of the delusion, were likely to have contributed to the form and content of MFs delusion of misidentification. In addition, our work with MF raises the possibility that the processing of face identity and facial expression are not as independent as previously proposed in cognitive models of face processing.

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Manuscript received 12 February 2001 Revised manuscript received 7 September 2001

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Ekman, P., & Friesen, W.V. (1976). Pictures of facial affect. Palo Alto, CA: Consulting Psychologists Press. Ellis, H.D., & Young, A. (1990). Accounting for delusional misidentifications. British Journal of Psychiatry , 157 , 239248. Ellis, H.D., Young, A.W., Quayle, A.H., & de Pauw, K.W. (1997). Reduced autonomic responses to faces in Capgras delusion. Proceedings of the Royal Society, London (B), 264, 10851092. Etcoff, N.L. (1984). Selective attention to facial identity and facial emotion. Neuropsychologia , 22, 281295. Gray, J.M., Young, A.W., Barker, W.A., Curtis, A., & Gibson, D. (1997). Impaired recognition of disgust in Huntingtons disease carriers. Brain, 120 , 20292038. Hirstein, W., & Ramachandran, V.S. (1997). Capgras syndrome: A novel probe for understandin g the neural representation of the identity and familiarity of persons. Proceedings of the Royal Society London, Series B, 264, 437444. Ivnik, R.J., Malec, J.F., Smith, G.E., Tangalos, E.G., & Kurland, L.T. (1992a). Mayos Older Americans Normative Studies: WAIS-R norms for ages 56 to 97. The Clinical Neuropsychol ogist, 6 (supplement), 130. Ivnik, R.J., Malec, J.F., Smith, G.E., Tangalos, E.G., & Peterson, R.C. (1996). Neuropsychologica l test norms above age 55: COWAT, BNT, MAE Token, WRAT-R Reading, AMNART, STROOP, TMT and JLO. The Clinical Neuropsychologis t, 10(3), 262278. Ivnik, R.J., Malec, J.F., Smith, G.E., Tangalos, E.G., Peterson, R.C., Kokmen, E., & Kurland, L.T. (1992b). Mayos Older Americans Normative Studies: WMS-R norms for ages 56 to 94. The Clinical Neuropsychologis t, 6 (supplement), 4982. Langdon, R., & Coltheart, M. (2000). The cognitive neuropsycholog y of delusions. Mind and Language , 15(1), 183216. Malec, J.F., Ivnik, R.J., Smith, G.E., Tangalos, E.G., Peterson, R.C., Kokmen, E., & Kurland, L.T. (1992). Mayos Older Americans Normative Studies: Utility of corrections for age and education for the WAIS-R. The Clinical Neuropsychologis t, 6 (supplement), 3147. Raven, J.C. (1947). Coloured progressive matrices sets A, Ab, B. London: H.K. Lewis. Sprengelmeyer , R., Young, A.W., Calder, A.J., Karnat, A., Lang, H.W., Homberg, V., Perrett, D.I., & Rowland, D. (1996). Loss of disgust: Perception of faces and emotions in Huntingtons disease. Brain, 119, 16471665. Sprengelmeyer , R., Young, A.W., Sprengelmeyer , A., Calder, A.J., Rowland, D., Perrett, D., Homberg, V., & Lange, H. (1997). Recognition of facial expressions: Selective impairment of specific emotions in Huntingtons disease. Cognitive Neuropsycholog y, 14, 839879. Stone, T., & Young, A.W. (1997). Delusions and brain injury: The philosophy and psycholog y of belief. Mind and Language, 12(3/4), 327364. Tranel, D., Damasio, H., & Damasio, A.R. (1995). Double dissociation between overt and covert face recognition. Journal of Cognitive Neuroscience, 7(4), 425432. Warrington, E.K. (1984). Recognition Memory Test. Windsor, UK: NFER-Nelson. Wechsler, D. (1981). Wechsler Adult Intelligence ScaleRevised. San Antonio: Psychological Corporation. Wechsler, D. (1987). Wechsler Memory ScaleRevised. San Antonio: Psychological Corporation. Young, A.W. (1998). Face and mind. New York: Oxford University Press. Young, A.W., Hellawell, D.J., van de Wal, C., & Johnson, M. (1996). Facial expression processing after amygdalotomy . Neuropsychologia , 34, 3139. Young, A.W., Newcombe, F., De Haan, E.H.F., Small, M., & Hay, D.C. (1993). Face perception after brain injury: selective impairments affecting identity and expression. Brain, 116, 941959.

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APPENDIX 1
Demographic details for control subjects and patient MF

Control subject RW HR RT FB FC Mean 69 67 70 69 69 69 yrs, yrs, yrs, yrs, yrs, yrs, 2 7 4 2 9 1

Age months months months months months month

Yrs education 16 16 15 16 13 15.2 years 17 years

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Patient MF

68 yrs, 5 months

APPENDIX 2
The four stories from the experimental Plausible/implausible reasoning test are listed below. The stories were presented one at a time and the subject was given as much time as he or she needed to read the story and answer the question for each story. They were required to circle the response they thought was correct.

Story 1
Fred lay still all the time. His wife and daughter tried to talk to him. Fred did not respond. Fred said that he was dead. Do you think Fred is dead? (Please Circle) YES NO CANT TELL

Story 2
Margaret thought that people were always following her around. She thought that the people following her around were people that she knew but that they were in disguise. It did not matter what time of day or night she left the house, the people would always follow her. She said that sometimes the disguises were so good that a young woman could be disguised as a very old stooped man with wrinkles and a bald head. Nobody ever saw these people, even when they were with Margaret. Margaret had contacted the police but after a long investigation they were unable to find any evidence of people in disguise following Margaret. Do you think people are following Margaret around? (Please Circle) YES NO CANT TELL

Story 3
Peter and his wife had been married for 30 years and had two children. One day Peter confronted the woman next to him in his bed and said that she was not his wife. Peter said the woman was an impostor.

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His children disagreed, and said that the woman in their home was his wife and their mother. His relatives also disagreed with him, and said that the woman was his wife. Peter agreed that his wife and this impostor looked very similar. He had no explanation as to where his wife was or where this impostor had come from. He continued to live with this woman in his house. Do you think the woman living with Peter is his wife? (Please Circle) YES NO CANT TELL

Story 4
Jack went to town to do some shopping. He paid for his groceries and then looked out of the shop window. Across the street he saw his wife Mary kissing another man. Jack rushed out onto the street but the couple were gone. He immediately phoned Mary at home. Mary answered the phone and denied leaving their home that day. Mary has a twin sister. Did Jack see his wife Mary kissing another man? (Please Circle) YES NO CANT TELL

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APPENDIX 3
Face expression recognition: Control data

Ekman & Friesen Percentage recognition rates Happiness Disgust Surprise Fear Sadness Anger 99.10 93.10 90.70 89.50 89.70 89.50 sd sd sd sd sd sd 2.51 5.20 7.78 5.91 7.87 11.39

Calder et al. n = 10 Mean identification rates 9.90 9.00 8.50 8.60 8.70 7.70 sd sd sd sd sd sd 0.32 1.25 1.58 1.17 1.34 1.42

Our controls n = 5 Mean identification rates 9.8 9.3 9.2 8.2 8.2 8.2 sd sd sd sd sd sd 0.45 0.55 1.09 1.30 1.64 1.30