An odd manifestation of the Capgras syndrome: Loss of familiarity even with the sexual partner

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An odd manifestation of the Capgras syndrome: Loss of familiarity even with the sexual partner
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  This article appeared in a journal published by Elsevier. The attachedcopy is furnished to the author for internal non-commercial researchand education use, including for instruction at the authors institutionand sharing with colleagues.Other uses, including reproduction and distribution, or selling orlicensing copies, or posting to personal, institutional or third partywebsites are prohibited.In most cases authors are permitted to post their version of thearticle (e.g. in Word or Tex form) to their personal website orinstitutional repository. Authors requiring further informationregarding Elsevier’s archiving and manuscript policies areencouraged to visit:  Author's personal copy Neurophysiologie Clinique/Clinical Neurophysiology (2008)  38 , 177—182 Disponible en ligne sur journal homepage: ORIGINAL ARTICLE/ARTICLE ORIGINAL An odd manifestation of the Capgras syndrome:Loss of familiarity even with the sexual partnerUne manifestation singulière du syndromede Capgras: la perte de familiarité pourle partenaire sexuel C. Thomas Antérion ∗ , P. Convers, S. Desmales, C. Borg, B. Laurent Neuropsychology Unit, Neurology Department, Bellevue Hospital, CHU de Bellevue, 25,boulevard Pasteur, 42055 Saint-Étienne, France Received 14 March 2008; accepted 12 April 2008Available online 23 April 2008 KEYWORDS Capgras syndrome;Implicit memory;Recognition;Familiarity;Emotion;Sexual affect;Lewy body disease Summary  We report the case of a patient who presented visual hallucinations and identifi-cation disorders associated with a Capgras syndrome. During the Capgras periods, there wasnot only a misidentification of his wife’s face, but also a more global perceptive and emotionalsexual identification disorder. Thus, he had sexual intercourse with his wife’s ‘‘double’’ withouthaving the slightest recollection feeling of familiarity towards his ‘‘wife’’ and even changed hissexual habits. To the best of our knowledge, he is the only neurological patient who made hiswife a mistress. Starting from this global familiarity loss, we discuss the mechanism of Capgrasdelusion with reference to the role of the implicit system of face recognition. Such behaviorof familiarity loss not only with face but also with all intimacy aspects argues for a specificdisconnection between the ventral visual pathway of face identification and the limbic systeminvolved in emotional and episodic memory contents.© 2008 Elsevier Masson SAS. All rights reserved. MOTS CLÉS Syndrome deCapgras ;Mémoire implicite ;Reconnaissance ;Familiarité ; Résumé  Nous rapportons le cas d’un patient présentant des hallucinations visuelles et destroubles de l’identification dans le cadre d’un syndrome de Capgras. Durant les périodes symp-tomatiques, il existait non seulement un trouble d’identification du visage de son épouse, maiségalementundésordreperceptifetémotionnel,plusglobald’identificationsexuelledecelle-ci.Ainsi, il avait des relations sexuelles avec le  « doublede son épouse » , sans éprouver le moin-dre sentiment de familiarité par rapport à des relations antérieures avec sa  « propre épouse » .Il en résulta une modification des habitudes sexuelles lors des relations avec le  « double » . Ànotre connaissance, il s’agit du seul patient neurologique qui fit de son épouse une maîtresse! ∗ Corresponding author. E-mailaddress: (C. Thomas Antérion).0987-7053/$ – see front matter © 2008 Elsevier Masson SAS. All rights reserved.doi:10.1016/j.neucli.2008.04.003  Author's personal copy 178 C. Thomas Antérion et al. Partant de ce cas, nous proposons d’interpréter les mécanismes à la base de la délusion deCapgras en référence au système de reconnaissance implicite des visages. Ce phénomènede perte de familiarité concernant non seulement le visage, mais également tous les aspectsde la vie intime suggère une dysconnection entre les voies visuelles ventrales d’identificationdes visages et le système limbique impliqué dans le souvenir de leur contenu émotionnel et deleur association à des épisodes antérieurs de la vie.Émotion ;Affects sexuels ;Maladie des Corps deLewy© 2008 Elsevier Masson SAS. All rights reserved. Introduction Hallucinations are observed during the evolution of sev-eral neurodegenerative disorders, particularly in Parkinson’sdisease (PD), Alzheimer’s disease (AD) and Lewy body dis-ease (LBD) [2,4,15]. However, there have been few specific studies on the phenomenology of hallucinations. Thus, itis commonly reported that hallucinations are usually visualand complex, and variably consist of characters, animals orobjects. Visual hallucinations occurring in LBD differ fromthose occurring in AD in that they appear much earlier in thedisease,aremorevariable,moreoftenassociatedwithhear-ing hallucinations, and much more persistent in time [3,15].Although patients are sometimes aware of the hallucinatorycharacter of these phenomena, their criticism may be par-tial, fluctuating or delayed: initially, the patient is oftenmisled by the realism of the hallucination. There are fre-quent reports on delirious ideas or identification/familiaritydisorders, particularly with the appearance of a Capgrassyndrome [7,8], i.e. not recognizing a relative who is thenmistaken for a double.WereportonapatientwithapossibleLBDwhodevelopedaclinicalpatternassociatingvisualhallucinationswithaFre-goli delusion (belief than unknown people are his closes)and, eventually, a Capgras syndrome (belief that a signifi-cant person has been replaced by an impostor). Here, thesignificant person was the wife who was mistaken for a dou-ble. The peculiarity of this observation is that the patienthad sexual intercourse with his double, with the familiaritydisorder remaining unchanged during their intimacy. As faras we know, from the neurological literature, he is the onlypatient who made his wife his mistress. Case report A 70 year-old right-handed ex-artisan (RP) had Parkinson’ssymptoms with bilateral motor symptoms and akinesia forsix months, when he developed visual hallucinations duringtwo weeks. As the extrapyramidal syndrome was not thatincapacitating, he had not received any treatment. He firstsaw his mother who had died twenty years before and wasdisappointed that she could not speak. This was a fluctuat-ing phenomenon, which lasted about a fortnight and thendisappeared for ever. It occurred anytime during the dayand was of a hallucinatory nature, the patient building hisimages from non visual delusions. While he was in hospi-tal for a check-up, he also presented hallucinatory episodesduring which he saw several women in front of him, whomhe identified as his wife. At the same time, he also experi-enced a delusion syndrome: he mistook a nurse for his wife,although there was no physical resemblance between both.These hallucinatory episodes were referred to as a Fregolisyndrome, even though he did not develop any delirious oraggressive ideas towards these ‘‘doubles’’, merely noticingthat it was very strange to see so many wifes at the sametime! This phenomenon occurred several times and alwaysin the presence of his wife.CT-scan disclosed a right temporal atrophy involving thepolar, external and internal parts (amygdala and hippocam-pus) of the temporal lobe (Fig. 1). SPECT with HMPAOdisclosed a perfusion decrease in the right frontal andtemporal lobes (Fig. 2). The neuropsychological evaluationsuggested impaired performance on frontal and visuospatialtests. The Mini Mental State Examination (MMSE) score was27/30[13].Hehadlowerscoresinvisualdiscriminationtasks and in tests of frontal functions. The main data are shownin Table 1. There were no difficulties for discriminating theemotional expressions of the Eckman faces expressing fear,happiness, surprise or disgust (Fig. 3). Even if RP had somedifficulties to identify some celebrities on photography, hedid not present prosopagnosia in real life as he easily recog-nized his family members and his close friends.Such clinical presentation associating a Parkinson’ssyndrome with profuse hallucinations, the profile of neu-ropsychological disorders and the fluctuating nature ofbehavioral symptoms led us to evoke a probable LBD [20].Three months later, the patient developed a Capgras syn-drome. He thought his wife was an impostor. Both his wifeand her double had the same given names, the same maidennames, very similar looks and manners. Both had brotherswho had the same ages and names. RP was unable to showthe differences of appearance between his wife and theimpostor and was very attentive to explore the most sub-tle aspects of her face, like a small spot on her forehead.He often found the double more aged than his real wife. Norationalization was able to induce correction, even if oneexplained that the impostor had the same clothes and jew-elsashiswife,orifhiswifetoldhimtheirfamilialhistoryandpersonal medical past. As commonly reported in literature,this phenomenon fluctuated during the day, the patient thenbegging his wife not to leave him alone with her ‘‘double’’.The peculiarity of this observation is that the wifereported that her husband had frequent sexual intercoursewith the imposter. Her husband then asked the ‘‘double’’not to say anything to his wife when she would come back.He never had any doubt about the person he found him-self with. His wife specified that he solicited her severaltimes in the afternoon, whereas, it was quite unusual forhim to do so. Above all, he was very kind to her and devel-oped preliminaries she was no longer used to. Therefore,she had the feeling he loved her like a man loves his mis-tress. Besides, he had new sexual practices, which he hadn’thad with her for nearly 45 years of conjugal life. Duringthe periods without misidentification he also had two more‘‘ordinary’’ intercourse with his ‘‘usual wife’’.  Author's personal copy An odd manifestation of the Capgras syndrome 179 Figure 1  CT scan: atrophy of the right temporal lobe: pole,amygdala, external cortex and hippocampus. Table 1  Neuropsychological performances of RP patient.Tests ScoresMMS 27Categorial fluency (two minutes)(animals)24Alphabetic fluency (two minutes) (p) 17Grober et Buschke verbal memory test(total recall)44/48Grober et Buschke (three free recalls)  16/48 Recognition 15/16Visual recognition (Benton)  0/15 Clock test  2/7  Thurstone test  3/30  DMS 48 (immediate pictures recognition)  36/48 DMS 48 (delayed pictures recognition)  34/48 Similitude (WAIS)  2/19  Stroop (words) 61Stroop (colors) 56Stroop (inhibition)  11(3 errors) Results shown in italics are below standard. These symptoms were observed for six weeks andeventually disappeared with transient neuroleptic treat-ment (risperdone 2mg/day) and long-term treatment withdonezepil. Discussion The Capgras delusion is the most common of the delusionalmisidentification syndromes (DMS) [7,8]. In this syndrome,well-known people are considered as being replaced byimpostors. The Fregoli syndrome, in which strangers areconsidered as significant people, is less common. Both syn-dromes could be associated to the same patient but thisrarely occurs. These delusions have been reported in bothpsychiatric and neurological literature [10,12,25]. In many studies, the Capgras syndrome has been considered either,as an example of the bridges between neurological andpsychiatric diseases or, as a window on face recognition.Neurological studies have focused on the importance offrontal and right hemisphere pathology [1,12], especially in degenerative disorders like AD, PD and more recently LBD[21,23]. The anatomical correlations are imprecise in ADand PD but more convincing in LBD where an atrophy of thetemporal and the frontal cortex, associated with brainstemlesions, induces a strong tendency to hallucinate. Horikawaet al., [17] reported a case of Capgras syndrome in interic-tal psychosis where the SPECT and HMPAO imaging revealedworsening of hypoperfusion in the entire right hemisphereafter onset of the psychotic symptoms and became normalafter the end of episode. The right-hemisphere prefer-ence is not constant, since Kanemoto [18] described aperi-ictal Capgras syndrome after a clustered ictal fearinduced by a depth stimulation of the left internal temporallobe.Many authors searched for a perceptive or integrativeimpairment that would be able to explain the misidentifi-cation syndrome, without convincing results. Some authorsdescribed difficulties to identify the facial expressions ofanger, disgust and fear, or to match faces across theseexpressions while the visual processing of identity, genderand age of familiar and unknown faces was intact [5]. Oth- ers emphasized the inability to judge gaze direction [16].These abilities were preserved in our case, in keeping withother cases of literature, which situates Capgras syndrome‘‘out of sight’’ [9]. Abnormalities of auditory P300, which is considered as an index of the on-line updating of work-ing memory, were described in a case of associated Capgrasand Fregoli syndrome [24]; however, the Capgras delusion is modality-specific and not auditory related. Indeed, as previ-ously noticed [16], our patient claimed that his wife was an impostor only when he was looking at her, but never whenspeaking to her on the telephone.More than elaborated sight impairment for persons,the Capgras syndrome seems to correspond to dissociationbetween what is remembered, which remains intact andfamiliarity, which is impaired. In that sense, Capgras con-stitutes a mirror picture of prosopagnosia, in which thereis an inability to explicitly identify faces, but familiarityis preserved as demonstrated by the persistence of skinconductance responses to familiar people. Some studiesanalyzing covert recognition for face processing with skinconductance focus on the implicit deficit in the Capgrassyndrome [16]: the case (DS) had his delusion with his par- ents and his skin conductance responses to photographsof familiar people, including his parents, were not largerin magnitude than to photographs of unfamiliar people.The authors [16] suggest that DS connections from face-processing areas in the fusiform gyrus to the limbic systemhave been damaged; therefore, owing to the absence oflimbic activation, DS creates separate memory ‘‘files’ ofthe same person and is unable to extract and link thecommon denominator of successive episodic memories con-  Author's personal copy 180 C. Thomas Antérion et al. Figure 2  SPECT with HMPAO: decreased perfusion in the right frontal and temporal regions. cerning the same person. Such absence of implicit responsetofamiliarwasreproducedinseveralotherscasesofCapgrassyndromes [11].Covert face recognition demonstrated by skin conduc-tance and behavioral indexes is mediated by differentpathways than those altered in prosopagnosia. We proposea dysconnection between the limbic system and the ven-tral pathways of face processing, the latter being intact inCapgras as opposed to prosopagnosia (Fig. 4). Therefore,the identified face does not activate all personal files ofmemories that are required for familiarity [26]. Functional MRI performed in normals viewing familiar and unfamiliarfaces or listening to familiar and unfamiliar voices, showedincreased neural activity in the posterior cingulate cortex,including the retrosplenial cortex. These changes occur irre-spective of stimulus modality, as opposed to the modules Figure 3  Emotion discrimination on the Eckman faces: from left to right neutral, happiness, fear, disgust and surprise.
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