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    This article was downloaded by: [Universidad de Chile]On: 25 June 2013, At: 06:46Publisher: RoutledgeInforma Ltd Registered in England and Wales RegisteredNumber: 1072954 Registered office: Mortimer House, 37-41

    Mortimer Street, London W1T 3JH, UK

    Cognitive

    NeuropsychiatryPublication details, including

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    The role of affect and

    reasoning in a patient

    with a delusion ofmisidentificationNora 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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    redistribution, reselling, loan, sub-licensing, systematic supply,or distribution in any form to anyone is expressly forbidden.

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    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

    Royal Prince Alfred Hospital and University of Sydney, Australia

    Max ColtheartMacquarie 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    false belief that someone has been replaced by an almost identical impostorpatient MF misidentified his wife as his former business partner.

    Method. Detailed investigation of MFs face processing, affective response andaffect 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 ofanger, disgust, and fear, or to match faces across expressions. MF also showed a

    reduced affective responsiveness to his environment, and impaired reasoningability.

    Conclusions. We propose that MFs delusion of misidentification resulted from a

    combination of affective deficits, including impairment of both affective responseand affect perception, in addition to an inability to evaluate, and reject, implausibleideas. 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. Inaddition, the results raise the possibility that the processing of face identity and

    facial expression are not as independent as previously proposed in models of faceprocessing.

    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: [email protected] u

    # 2002 Psychology Press Ltd

    http://www.tandf.co.uk/journals/pp/13546805.html DOI:10.1080/13546800143000203

    COGNITIVE NEUROPSYCHIATRY, 2002, 7 (2), 113137

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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, thepatient 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 describedby 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 varieddramatically 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

    A DELUSION OF MISIDENTIFICATION 115

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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 prosopagnosia are unable

    to recognise their own face in a mirror yet generally are not reported as havingthe 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 atthe 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 difficultiesand 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 encephaloma-

    lacic changes in the frontal lobes bilaterally, a small calcified subdural

    haematoma in the left frontal region, mild cerebellar atrophy, and a lacunarinfarct 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 neuropsychological

    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, althoughhe 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.

    A DELUSION OF MISIDENTIFICATION 117

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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 shehad 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 similarcolouring 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 thegastrostomy operation, he and his wife were in the small elevator in their multi-

    storey 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 ofthe 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 conductinginvolved 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 (includinga 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 non-

    verbal memory function. His verbal memory for stories and word associations

    was intact in the high average to superior range (WMS-R; Wechsler, 1987), as

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    TABLE 1

    Results of tests of cognitive ability for patient MF

    Tests Patient MF

    IQ NART (Estimated Full Scale IQ) 107 (Average)

    Language Test of Reception of Grammar (TROG) 73/80 (91%)

    WAIS-R MOANS ASS

    Vocabulary 8

    Mental Arithmetic 4

    Similarities 9

    Non-verbal skills Ravens Coloured Progressive Matrices 75%tile

    Clock drawing 10/10

    Copying of shapes, designs and bicycle intactCopy of 3-dimensional cube intact

    REY Complex Figure TestCopy 29.5*

    WAIS-R MOANS ASS

    Picture Completion 13

    Picture Arrangemen t 10

    Block Design 8

    Digit Symbol 4

    Attention/ WMS-R MOANS ASS

    Concentration Digit Span 5 (5 forward, 3 backward )

    Visual Span 9 (7 forward, 6 backward )

    Memory WMS-R MOANS ASS

    Logical Memory I 14

    Logical Memory II 14

    Verbal Paired Associates I 12

    Verbal Paired Associates II 13

    Visual Reproduction I 12Visual Reproduction II 9

    (Visual Reproduction II, with prompt) (12)

    Rey Complex Figuredelaye d recall 8.5*

    Autobiographica l Memory Interview Personal Sem Autobiograp h

    Childhood 15 borderline 7 Acceptable

    Early Adult Life 17 Acceptable 9 Acceptable

    Recent Life 21 Acceptable 6 Acceptable

    Total 53 Acceptable 22 Acceptable

    Warrington Recognition Memory Test Raw Score ASS

    Words 49 15

    Faces 41 9

    (Continued overleaf)

    A DELUSION OF MISIDENTIFICATION 121

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    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

    TABLE 1

    Continued

    Tests Patient MF

    Executive Function Trails A (ASS) 7

    Trails B incomplete

    Controlled Oral Word Association Test

    Total in 3 minutes (phonemic cue) 13 ASS 3 1st percentile

    Animal FluencyTotal in 1 minute 9 < 10th percentile

    Colour Form Sorting Ttest 2/2 categories

    Wisconsin Card Sorting Test

    Numbe r of categorie s complete d 0

    Errors 96 1st percentilePerseverative Errors 94 < 1st percentile

    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, Autobiograph Autobio-

    graphical).

    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 NormativeStudies (Malec et al., 1992; Ivnik et al., 1992a; Ivnik et al., 1992b; Ivnik et al., 1996). *within 1 sd of

    mean.

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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.

    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 unfamiliarfaces

    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 anapproximate 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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    people. The unknown faces were matched according to gender, age, and

    physical similarity. An additional 37 unknown 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

    TABLE 2

    Results for the face-processing tests

    MF Controls

    Face Matching Facial Recognitio n Test (Benton) 47 Range 4154

    Age Identification (Unfamiliar faces) Age (years) Range (years)

    1 15

    1 13

    18 1320

    12 38

    35 2045

    25 2535

    35 4455

    45 4560

    70 5575

    70 7084

    Face Recognitio n Personally familiar (family members ) MFs wife

    Identified as familiar 12/12 12/12

    Named 12/12 12/12

    Famous

    Identified as familiar 19/22 19/22

    Name/Specific semantic identification 17/22 18/22

    Unfamiliar (strangers faces)

    Identified as unfamiliar 56/59 56/59

    Facial Affect Ekman & Friesen Facial Affect Photos (/10) Controls (n = 5)

    Perception Happy 10 9.8 (sd 0.45)

    Sadness 8 8.2 (sd 1.64)

    Surprise 10 9.2 (sd 1.09)

    Anger 7 8.2 (sd 1.30)

    Disgust 7*** 9.3 (sd 0.55)

    Fear 5** 8.2 (sd 1.30)

    Controls (n = 5)

    X sd

    Matching Faces Neutral Expression 33* 38.0 (2.45)

    Across Expressions Same Expressions 25*** 38.8 (1.79)

    Different Expressions 23*** 37.2 (3.35)

    *significant at p < .025, **significant at p < .01, ***significant at p < .0001.

    The Calder et al. (1996) subset of Ekman and Friesen Pictures of Facial Affect was used.

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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.

    Experiment 4a: Identification of personally knownfaces

    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 implicatedin 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, hecorrectly 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 whowas 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.

    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 emotionshe 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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    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%

    Figure 2. Face expression recognition.

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    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 testwas 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 expres-

    sions). 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 face-

    matching 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

    Figure 3. Face constancy: Matching faces across expressions.

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    actions and distinguishing essential from irrelevant details (Picture Arrange-

    ment, ASS 10), and conceptual reasoning (ASS 9).

    Experiment 8: Affect-neutral reasoning test

    The Wisconsin Card Sorting Test was administered to assess MFs reasoningability 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.

    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). Thefour 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 Controls (n = 5)

    Subject 1 Subject 2 Subject 3 Subject 4 Subject 5

    Story 1 Cant tell No No No No No

    Story 2 Cant tell No No No No No

    Story 3 No Yes Yes Yes Cant tell Yes

    Story 4 Cant tell No No Cant tell No 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 differentemotions, 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 facialexpressions 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 hadbeen 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 facialexpressions, 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 differentinstitution), 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 himshowing 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 thatMF 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), thestudy 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 expressionanalysis 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, incombination 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.

    Manuscript received 12 February 2001

    Revised manuscript received 7 September 2001

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    A DELUSION OF MISIDENTIFICATION 135

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    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 1Fred 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 2Margaret 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.

    APPENDIX 1

    Demographic details for control subjects and patient MF

    Control subject Age Yrs education

    RW 69 yrs, 2 months 16HR 67 yrs, 7 months 16

    RT 70 yrs, 4 months 15

    FB 69 yrs, 2 months 16

    FC 69 yrs, 9 months 13

    Mean 69 yrs, 1 month 15.2 years

    Patient MF 68 yrs, 5 months 17 years

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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

    APPENDIX 3

    Face expression recognition: Control data

    Ekman & Friesen

    Percentage recognition

    rates

    Calder et al. n = 10

    Mean identification

    rates

    Our controls n = 5

    Mean identificatio n

    rates

    Happiness 99.10 sd 2.51 9.90 sd 0.32 9.8 sd 0.45

    Disgust 93.10 sd 5.20 9.00 sd 1.25 9.3 sd 0.55

    Surprise 90.70 sd 7.78 8.50 sd 1.58 9.2 sd 1.09

    Fear 89.50 sd 5.91 8.60 sd 1.17 8.2 sd 1.30Sadness 89.70 sd 7.87 8.70 sd 1.34 8.2 sd 1.64

    Anger 89.50 sd 11.39 7.70 sd 1.42 8.2 sd 1.30

    A DELUSION OF MISIDENTIFICATION 137

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