Coexistence of Capgras and Frégoli syndromes associated to frontotemporal volume reduction and cerebral white matter hyperintensities

Coexistence of Capgras and Frégoli syndromes associated to frontotemporal volume reduction and cerebral white matter hyperintensities

Coexistência das síndromes de Capgras e Frégoli associadas à redução de volume
frontotemporal e hiperintensidades em substância branca cerebral

Gizela Turkiewicz1, Marcus Vinicius Zanetti2, Stevin Zung3, Quirino Cordeiro4

1 Mestranda do Departamento de Psiquiatria da Faculdade de Medicina da Universidade de São Paulo (FMUSP).
2 Pesquisador do Laboratório de Neuroimagem e Psiquiatria (LIM-21) da FMUSP.
3 Pesquisador do Projeto Genética e Farmacogenética (ProGene) do Instituto de Psiquiatria do HC-FMUSP.
4 Pesquisador do Projeto Transtornos do Espectro Obsessivo-Compulsivo (Protoc) do Instituto de Psiquiatria do HC-FMUSP.

Recebido: 16/4/2009 – Aceito: 15/7/2009

Abstract

Background: Delusional misidentification syndromes are conditions in which the patients pathologically misidentify people, places, objects or events. They have been categorized in four subtypes: Capgras, Frégoli, intermetamorphosis and subjective double syndromes. Such syndromes may be present in patients with psychiatric disorders such as schizophrenia and mood disorders, and with neurological diseases such as Alzheimer, Parkinson and brain injury (trauma, vascular). Objectives: To describe and discuss a case of coexistent between Capgras and Frégoli syndromes in a female patient with paranoid schizophrenia and brain MRI findings.Methods: Psychiatric interview
and brain MRI scanning. Results: The patient presented structural magnetic resonance imaging periventricular and subcortical white matter hyperintensities on flair images mainly concentrated in the right frontotemporal region
and bilateral frontotemporal volume loss. Discussion: The described neuroimaging findings may represent an organic substrate to the delusional misidentification syndromes of the present case. The delusional symptoms in Capgras and Frégoli syndromes could be the result of a right temporolimbic-frontal disconnection which results in impossibility to associate previous memories to new information and consequently misidentifying symptoms. Moreover a volume loss of such cerebral regions, as observed in the present case, may also play a significant role in the development of delusional misidentification syndromes.

Turkiewicz G, et al. / Rev Psiq Clín. 2009;36(6):240-3

Key words: Schizophrenia, neuroimaging, magnetic resonance imaging, pathophysiology, psychosis.

Resumo

Contexto: Transtornos delirantes de identificação são condições nas quais os pacientes identificam de maneira patologicamente equivocada pessoas, lugares, objetos ou eventos. Esses transtornos têm sido categorizados em quatro diferentes subtipos: Capgras, Frégoli, intermetamorfose e síndrome do duplo subjetivo. Tais síndromes podem estar presentes em diferentes transtornos psiquiátricos, como esquizofrenia e transtornos do humor, bem como em diferentes doenças neurológicas, como Alzheimer, Parkinson, lesões cerebrais traumáticas ou vasculares. Objetivos: Descrever e discutir um caso de coexistência entre as síndromes de Capgras e Frégoli em uma paciente com esquizofrenia paranoide e com alterações cerebrais. Métodos: Entrevista psiquiátrica e ressonância magnética de crânio. Resultados: A paciente apresentava hiperintensidades periventriculares em aquisição flair e de substância branca subcortical concentradas principalmente na região frontotemporal direita, bem como perda do volume da região frontotemporal bilateral. Discussão: As alterações descritas podem representar substrato orgânico das síndromes dos transtornos delirantes de identificação. Os delírios nas síndromes de Capgras e Frégoli podem ocorrer como resultado de uma desconexão têmporo-límbica-frontal direita, resultando em uma impossibilidade de associar memórias prévias a novas informações, levando consequentemente a alterações na capacidade de reconhecimento. Ademais, uma perda do volume de tais regiões cerebrais também pode desempenhar papel importante no desenvolvimento de tais síndromes delirantes de identificação.

Turkiewicz G, et al. / Rev Psiq Clín. 2009;36(6):240-3

Palavras-chave: Esquizofrenia, neuroimagem, ressonância magnética, fisiopatologia, psicose.

Introduction

Delusional misidentification syndromes (DMS) are conditions in which the patients pathologically misidentify people, places, objects or events. DMS are categorized in four subtypes: Capgras, Frégoli, intermetamorphosis and subjective double syndromes1.

Memory impairment, executive dysfunction and perception abnormalities – mainly regarding facial recognition processes – are related to DMS. These syndromes were previously associated to brain structure abnormalities as cortical volume loss, mainly in frontal and temporal lobes, with right hemisphere predominance1.

Capgras syndrome is the delusional belief that a significant person has been replaced by a double (“an impostor”), who is psychologically different but physically identical to the person. Capgras syndrome is the most prevalent of DMS and it is associated to psychiatric disorders as schizophrenia and mood disorders, and to neurological diseases as Alzheimer, Parkinson and brain injury (trauma, vascular). Frégoli syndrome is the delusional belief that someone known is pretending to be someone else, taking the appearance of the other. Intermetamorphosis syndrome is characterized as changes in both the identity and the appearance of the misidentified person, exchanging identities with each other. The subjective doubles syndrome is a person’s belief of having an identical double1.

Herein we report a case of coexistent between Capgras and Frégoli syndromes in a patient with paranoid schizophrenia presenting structural magnetic resonance imaging (MRI) periventricular and subcortical white matter hyperintensitites (WMH) on flair images mainly concentrated in the right frontotemporal region and bilateral frontotemporal volume loss.

Case report

A 62-year-old right- handed woman was brought to a psychiatric hospital due to aggressive behavior associated to paranoid and misidentification symptoms. At the age of 44, she had developed persecutory delusions and auditory hallucinations, receiving the diagnosis of paranoid schizophrenia. She had no previous reports of alcohol or drug abuse nor mood or psychotic psychopathology.

About six months prior to her admission, she started to present a delusional belief that her 20-years-old son had been replaced by an impostor, physically familiar but psychologically different, who had came into her home to poison her and to kidnap her daughter. She believed that her 21-year-old daughter was actually 9 years-old and needed her protection from the impostor’s threat. This belief made her hostile to his son. During her inpatient period she misidentified her doctor with her husband. She insisted that the doctor was her husband disguised with a woman’s mask. Her husband had died two years before this time.

Brain MRI scanning revealed mild bilateral frontotemporal volume loss and the presence of hyperintensity lesions on flair images in both periventricular and deep white matter with greater burden in right hemisphere (Figure 1).

The patient was screened for cerebrovascular risk factors as arterial hypertension, diabetes, dyslipidemia, tabagism and none of them were positive.

Discussion

The present patient had the diagnosis of paranoid schizophrenia with starting symptoms at the age of 44. Late-onset schizophrenia is defined as illness onset after the age of 40, representing around 20% proportion of patients with schizophrenia diagnosis2. However, brain imaging findings in patients with schizophrenia seem to be similar regardless of the age of onset: higher ventricle-to-brain ratio, higher third ventricle volume and volume reductions of the left temporal lobe or superior temporal gyrus3,4. The MRI of the present patient revealed WMH in right anterior hemisphere and bilateral frontotemporal volume reduction. These findings may represent a specific organic substrate to the DMS psychopathological presentation of the case and will be discussed bellow.

The prevalence of Capgras syndrome in schizophrenia has been reported to be around 15%1. Frégoli syndrome is less common in patients with schizophrenia with its prevalence undetermined in such population. Though it is uncommon, the coexistence of both DMS in the same subject has been previously reported and suggests a common pathogenic basis for these syndromes1. Reports of DMS associated to brain injury or neurological conditions have led to the investigation of the neuroanatomic basis to these conditions5. DMS have been associated to bilateral cortical volume loss with right hemisphere predominance, involving frontal, parietal and temporal lobes so these syndromes have been classified as “right hemisphere damage delusions”6.

Right hemisphere plays a dominant role in facial perception in right-handed subjects. Previous authors have suggested that impairment in facial recognition plays a role in the pathogenesis of DMS6. Some studies have demonstrated impairment in judging facial emotions in patients with the diagnosis of schizophrenia associated to a global impairment in social skills which results from a right hemisphere dysfunction. However, in DMS there is a more specific impairment in facial recognition, which is also associated to right hemisphere alterations6. The delusional symptoms in Capgras syndrome could be the result of a right temporolimbic-frontal disconnection which results in impossibility to associate previous memories to new information and consequently misidentifying symptoms1. These pathways, connecting limbic structures to other brain regions, provide the sense of familiarity and affective significance3. Frégoli syndrome has also been associated to impairment in face processing1. Facial processing involves right ventromedial occipito-temporal regions and areas of prefrontal cortex via the uncinate fasciculus and limbothalamic pathways6.

Previous reports have been largely restricted to grey matter, and as far as we know, there was only one previous report of WMH lesions affecting the brainstem in DMS6. The presence of deep WMH suggests that DMS could result from a disruption of connections involving frontal lobe to other cortical and subcortical areas. These findings support previous reports that DMS comes from the disconnection of face processing areas in the inferior temporal lobes from limbic system6. Thus deep frontal lesions could disconnect temporal and limbic regions from the frontal lobe which could result in a disturbance of familiarity of people and places1.

WMH are frequent neuroimaging findings in the elderly population, with a prevalence of up to 100% in the literature7,8. Nevertheless, the burden and severity of WMH are greater in individuals with vascular risk factors9. However, such risk factors were not found in this case, which illustrates that WMH could be part of an aging process or a silent disease that results in physical and psychological impairment. In the present case there were deep WMH lesions greater than 6 mm in diameter, with extensive periventricular damage and presence of infratentorial hyperintensities, fulfilling all the three Fazekas et al.10 criteria of WMH severity, corroborating the hypothesis that in this case report the cerebral lesions were more pronounced than those usually found in association with normal aging.

White matter lesions have been particularly studied in subjects suffering from mood disorders and the fact that WMH may appear even in children and adolescents, together with the finding of no correlation between the presence of WMH and cerebrovascular disease in adult patients with bipolar disorder suggest that a mechanism different from ischemia might operate in such cases11,12. Future histopathological studies are warranted to test the hypothesis that WMH associated to DMS are ischemic in origin11. Thus the
WMH could contribute for the misidentification symptoms in this patient.

Moreover the WMH lesions, the patient also presented a MRI bilateral frontotemporal volume loss. Recent neuroimaging findings have evidenced frontotemporal structural volume reduction over time in the clinical course of patients with schizophrenia13. According to anatomical findings in patients with Alzheimer’s disease, frontotemporal atrophy, predominantly in the right hemisphere, may also play a significant role in the development of DMS1.

As there was a large time interval between the installation of schizophrenia and DMS, it may lead us to think of a posterior installation of the brain injury which caused the symptomatology of DMS. This case report speculates that DMS may be associated with the progression of frontotemporal atrophy and WMH installation in late life. However, we are aware prospective follow-up studies, with serial MRI scans measuring frontotemporal volume reduction and WMH volume changes over time in correlation with DMS, is necessary to confirm such hypothesis.

Referências

1. Feinberg TE, Roane DM. Delusional misidentification. Psychiatr Clin N Am. 2005;28:665-83.
2. Howard R, Rabins PV, Seeman MV, Jeste DV, The international late-onset schizophrenia group. Late-onset schizophrenia and very-late-onset schizophrenia-like psychosis: an international consensus. Am J Psychiatry. 2000;157:172-8.
3. Sallett PC, Gattaz WF. Estudos de neuroimagem na classificação de Leonhard. Rev Psiq Clín. 2002;29:237-47.
4. Araripe Neto AGDA, Bressan RA, Busatto GF. Physiopathology of schizophrenia: current aspects. Rev Psiq Clín. 2007;34:198-203.
5. Alcoverro O, Sierra AC. Síndrome de Capgras. Actas Esp Psiquiatr. 2001;29:70-2.
6. Edelstyn NMJ, Oyebode F, Barrett K. The delusions of Capgras and intermetamorphosis in a patient with right-hemisphere white-matter pathology. Psychopathology. 2001;34:299-304.
7. Sachdev PS, Wen W, Christensen H, Jorm AF. White matter hyperintensities are related to physical disability and poor motor function. J Neurol Neurosurg Psychiatry. 2005;76:362-7.
8. Ávila R, Bottino CMC. Cognitive alterations update among elderly with depressive syndrome. Rev Psiq Clín. 2006;28:316-20.
9. Awad IA, Spetzler RF, Hodak JA, Awad CA, Carey R. Incidental subcortical lesions identified on magnetic resonance imaging in the elderly: I. Correlation with age and cerebrovascular risk factors. Stroke. 1986;17:1084-9.
10. Fazekas F, Offenbacher H, Fuchs S, Schmidt R, Niederkorn K, Horner S, et al. Criteria for an increased specificity of MRI interpretation in elderly subjects with suspected multiple sclerosis. Neurology. 1988;38:1822-5.
11. Zanetti MV, Cordeiro Q, Busatto GF. Late onset bipolar disorder associated with white matter hyperintensities: a pathophysiological hypothesis. Prog Neuropsychopharmacol Biol Psychiatry. 2007;31:551-6.
12. McDonald WM, Tupler LA, Marsteller FA, Figiel GS, DiSouza S, Nemeroff CB, et al. Hyperintense lesions on magnetic resonance images in bipolar disorder. Biol Psychiatry. 1999;45:965-71.
13. Hirayasu Y. Brain imaging in schizophrenia. Neuropathology. 2007;27:601-3.

Prim Care Companion CNS Disord. 2012; 14(1): PCC.11l01227.

Published online 2012 January 26. doi:  10.4088/PCC.11l01227

PMCID: PMC3357570

A “Contemporary” Case of Frégoli Syndrome

Tanya Kochuparackal, MD and Andor E. Simon, MD

Author information ► Copyright and License information ►

To the Editor: Frégoli syndrome belongs to the group of delusional misidentification syndromes and was first described in 1927.1 The hallmark of Frégoli syndrome is the belief that a familiar person is disguised as a strange person, ie, the familiar person has taken on a different physical appearance but remains the same person psychologically. The syndrome has been associated with organic cerebral dysfunction, in particular of the right hemisphere; however, most cases occur in the setting of schizophrenia.2,3 We report the case of a patient with schizophrenia who developed Frégoli syndrome within the context of using social networking Web sites.

Case report. Mr A, a 21-year-old man, was referred to our psychiatric outpatient service in 2010 with a first episode of schizophrenia (DSM-IV criteria). He had developed the belief that his daily facial cream strongly attracted female students and claimed that this cream was able to perfect his facial look. He spent an excessive amount of time on Facebook, where he met a young woman with whom he wished to engage in an intimate relationship, but the young woman withdrew. Subsequently, he developed the belief that each time he was contacted by other young women via Facebook, it was in fact the young woman with whom he had been in contact before and who now was disguising herself. He strongly believed that she was applying the same cream as he did himself to transform her facial looks. Thus, he was convinced that, despite her withdrawal, she was actually interested in pursuing a relationship with him. Full neurologic assessments, including cerebral magnetic resonance imaging, revealed no abnormalities.

Frégoli syndrome has been associated with some degree of objective face-processing impairment.3,4 It is understood as a syndrome of hyperidentification caused by a breakdown of the identification process, thus leading to the inability to attribute uniqueness to a specific person.2 As in the present case, hyperidentification may not be limited to one single person only.2 This is the first case reporting emergence of Frégoli syndrome in association with using social network Web sites. As these networks generally feature portraits of familiar persons and strangers alike and are massively used today throughout the world to initiate and foster social contacts, the combination with Frégoli syndrome is intriguing. To what extent this shift in social behavior will be paralleled by a shift of context in which this disorder occurs remains an open yet tempting question that may be answered by further reports and case studies of Frégoli syndrome.

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References

1. Courbon P., Fail G. Syndrome d'llusion de Frégoli et schizophrenie. Bull Soc Clin Med Ment.1927;15:121–124.

2. Christodoulou G.N., Margariti M., Kontaxakis V.P., et al. The delusional misidentification syndrome: strange, fascinating, and instructive. Curr Psych Rep. 2009;11(3):185–189. [PubMed]

3. Walther S., Federspiel A., Horn H., et al. Performance during face processing differentiates schizophrenia patients with delusional misidentifications. Psychopathology. 2010;43(2):127–136.[PubMed]

4. Ellis H.D., Young A.W. Accounting for delusional misidentifications. Br J Psychiatry.1990;157(2):239–248. [PubMed]

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Articles from The Primary Care Companion for CNS Disorders are provided here courtesy of Physicians Postgraduate Press, Inc.

AN IMPOSTOR IN THE FAMILY ARTICLE #252 • WRITTEN BY GERRY MATLACK

Imagine, 


imagine, if you will, that one by one your friends and family-- the people closest to you-- are being removed and replaced with exact duplicates. Although they are identical in appearance and manner, you are certain that these people are not your loved ones. They are impostors. While most people would become deeply paranoid in such a scenario, there are some individuals who experience such things every day without fear... and just wonder, "why?" Such is the life of people stricken with Capgras' Syndrome.

A person with Capgras' Syndrome suffers from the delusion that one or more of their close friends or family members have been replaced with exact duplicates, and they cannot be shaken from this belief in spite of an otherwise clean bill of mental health. In some instances, the person believes that they themselves are, in whole or in part, a duplicate. Unlike the paranoia expected from such a condition, there is never a motive assigned for the appearance of the duplicates - the patients do not believe someone is "out to get them," but they are at a loss for an explanation why anyone would want to replace their loved ones.

This odd misperception is named after the French psychiatrist Jean Marie Joseph Capgras, who described the case of a Madame M. in 1923. The woman insisted that identical-looking persons had taken the place of her family. Over time her delusion expanded to include neighbors, friends and acquaintances. But Madame M. never bothered to get to know these impostors because it was her belief that each one regularly left to make room for the next double. In all, she eventually claimed to have had more than eighty husbands.

People suffering from Capgras' Syndrome can sometimes even doubt their own identity after seeing their reflection in a mirror. One man pinched himself on the arm after seeing his reflection at the doctor's office, and wondered aloud whether he and the man in the reflection were the same person. There was also a woman who flew into a jealous rage every time she caught sight of her own reflection, believing this "other woman" was trying to lure her husband away from her. Her husband eventually covered every reflective surface in the house in an effort to keep her from hurting herself. Oddly enough, she had no problem recognizing herself in the mirror of her makeup compact, but anything larger resulted in an assault on the imaginary impostor. Her doctor tried a novel solution: he gathered a number of mirrors of varying sizes, and had the woman view herself in each one. He started with the smallest and gradually moved to the next larger as soon as she recognized herself. Ultimately she was able to see herself in a full-length mirror, and she was cured from then on.

In some instances, individuals with the Capgras delusion see duplicate objects rather than duplicate people. One doctor reported a patient who believed that his poodle had been replaced with an identical dog, and another reported a patient who believed that during the night his running shoes and many other personal possessions were being replaced.

Capgras' delusion always centers around just one of the subject's senses. The most common is the sense of sight; for example, one person readily recognized his wife on the phone when speaking to her, yet when she arrived in the flesh he thought the impostor was actually his sister-in-law. Blind people have also been diagnosed with the disorder, and they believe that the voices of certain loved ones are actually coming from duplicates.

While the causes of Capgras' syndrome are not specifically known, there is no shortage of theories. It has been shown that many people with the syndrome have brain lesions in the right temporal lobe from traumatic injuries, epilepsy, and other causes, yet there are also significant numbers of patients with no such damage in evidence. Also, there is a somewhat higher incidence of schizophrenia among people with Capgras, and in New Zealand there is a markedly higher incidence of the disorder among the Maori people than in the general population.

Some earlier researchers attempted to draw connections to Prosopagnosia, a condition which prevents some people from being able to recognize faces. By measuring a person's galvanic skin response-- the amount of electrical resistance in the skin-- scientists can detect when an individual is experiencing emotions. Patients with Prosopagnosia show an emotional response to familiar faces, though they exhibit no conscious recognition. With a Capgras patient there is no such reaction. Though no emotional connection is present when shown a picture of their father, the patient will remark on the striking resemblance. This test also rules out mental illness as a definitive cause, since the emotional center of the brain would subconsciously react even with impaired perceptions.

Another proposed cause involves some form of damage or impairment in two lobes of the brain: One site of damage affecting the emotional connections with respect to people's faces, and the other affecting the brain's consistency-checking abilities.

In at least one case, doctors have successfully cured Capgras' Syndrome by suspending a prescription of diazepam, yet in other cases symptoms have disappeared after administering anti-psychotic medication. To date, no single treatment has been found to be consistently effective, and so far there is no single theory that can explain all the reported cases of Capgras' Syndrome. We do know, however, that the human mind uses many interlocking cognitive tricks to fill in the gaps of our observations, essentially building a simulation which allows us to interact with our world and society. When just one or two of those links go awry, the true complexity of that simulation is revealed by the fascinating problems that arise.

Written by Gerry Matlack, posted on 05 February 2007. Gerry is a contributing editor for DamnInteresting.com.