Prevalence of Capgras syndrome in Alzheimer's patients: A systematic review and meta-analysis / Prevalência da síndrome de capgras em pacientes com doença de alzheimer: uma revisão sistemática e metanálise

ABSTRACT The association between Capgras syndrome and Alzheimer's disease has been reported in several studies, but its prevalence varies considerably in the literature, making it difficult to measure and manage this condition. Objective: This study aims to estimate the prevalence of Capgras syndrome in patients with Alzheimer's disease through a systematic review, and to review etiological and pathophysiological aspects related to the syndrome. Methods: A systematic review was conducted using the Medline, ISI, Cochrane, Scielo, Lilacs, and Embase databases. Two independent researchers carried out study selection, data extraction, and qualitative analysis by strictly following the same methodology. Disagreements were resolved by consensus. The meta-analysis was performed using the random effect model. Results: 40 studies were identified, 8 of which were included in the present review. Overall, a total of 1,977 patients with Alzheimer's disease were analyzed, and the prevalence of Capgras syndrome in this group was 6% (CI: 95% I² 54% 4.0-8.0). Conclusion: The study found a significant prevalence of Capgras syndrome in patients with Alzheimer's disease. These findings point to the need for more studies on the topic to improve the management of these patients.

RESUMO A associação da síndrome de Capgras com a doença de Alzheimer é relatada em diversos estudos, porém a sua prevalência varia consideravelmente entre bibliografias, dificultando a real mensuração e manejo desta patologia. Objetivo: O objetivo deste estudo foi estimar, através de uma revisão sistemática, a prevalência da síndrome de Capgras em pacientes com a doença de Alzheimer, bem como revisar os aspectos etiológicos e fisiopatológicos relacionados à síndrome. Métodos: Uma revisão sistemática foi realizada utilizando-se as seguintes bases de dados: Medline, ISI, Cochrane, Scielo, Lilacs e Embase. A seleção dos estudos, extração de dados e análise qualitativa foi feita por dois pesquisadores de forma independente e seguindo rigorosamente a mesma metodologia, sendo as discordâncias resolvidas por consenso. A metanálise foi calculada utilizando-se modelo de efeito randômico. Resultados: Foram recuperados 40 estudos, dos quais 8 foram incluídos na presente revisão. Considerando todos os estudos incluídos, 1977 pacientes com doença de Alzheimer foram analisados, sendo encontrada a prevalência da síndrome de Capgras dentre esses pacientes de 6% (IC: 95% I² 54% 4,0-8,0). Conclusão: A presente revisão encontrou uma prevalência significativa da síndrome de Capgras nos pacientes com doença de Alzheimer. Tal achado implica na necessidade de mais estudos visando melhorar o manejo desses pacientes.

Síndrome de Capgras: una revision de la literatura / Capgras syndrome: a review of the literature

Los síndromes de falsa identificación delirante (DMS, por su sigla en inglés) son trastornos neuropsiquiátricos poco frecuentes que se caracterizan por tener ideas delirantes respecto a la propia identidad y la de otras personas, animales o lugares conocidos por el paciente. Los principales DMS son el Síndrome de Capgras (SC), el de Fregoli, el de doble subjetivo y el de intermetamorfosis. Se presentan en contexto tanto de enfermedades psiquiátricas como en cuadros orgánicos. Distintos modelos han tratado de encontrar una explicación a los DMS, con aportes tanto desde la psicología como de las neurociencias. Entre las causas están enfermedades neurodegenerativas, cuadros psiquiátricos, alteraciones estructurales, efecto de drogas, y alteraciones metabólicas. El manejo depende de la patología de base y las características clínicas específicas. Esta revisión se centra específicamente en el SC, ya que dentro de los DMS, es el más frecuente y estudiado

Delusional misidentification syndromes (DMS) are rare neuropsychiatric disorders that are characterized by having delirious ideas regarding one's and other people, animals or places identity known by the patient. The main DMS are the Capgras syndrome, the Fregoli syndrome, the subjective double syndrome and the intermetamorphosis syndrome. They appear in context of both psychiatric illnesses and organic disorders. Different models have tried to find an explanation to the DMS, with contributions from both psychology and neurosciences. Among the causes are neurodegenerative diseases, psychiatric symptoms, structural alterations, drug effects, and metabolic alterations. Management depends on the underlying pathology and the specific clinical characteristics. This review focuses specifically on the SC, since within the DMS, it is the most frequent and studied.

Capgras syndrome associated with limbic encephalitis in a patient with diffuse large B-cell lymphoma / Síndrome de Capgras associada a Encefalite Límbica em um Paciente com Linfoma Difuso de Grandes Células B

We report the case of a patient with insidious onset and slowly progressive cognitive impairment, behavioral symptoms, temporal lobe seizures and delusional thoughts typical of delusional misidentification syndromes. Clinical presentation along with extensive diagnostic work-up revealed limbic encephalitis secondary to diffuse large B-cell lymphoma. The patient underwent immunotherapy with high-dose corticosteroid but no significant improvement was observed. No specific treatment for lymphoma was performed because the patient died of septic shock following a nosocomial respiratory infection. Delusional misidentification syndromes are an unusual and unique form of cognitive impairment in which a patient consistently misidentifies persons, places, objects, or events. Capgras syndrome is the most common subtype of this disorder, being defined by the recurrent and transient belief that someone close has been substituted by an imposter. These entities are generally associated with neurodegenerative diseases and psychiatric disturbances. Rare reports of associations between misidentification syndromes and autoimmune diseases such as multiple sclerosis have been published, but no papers address a correlation with limbic encephalitis or lymphoma.

Nós relatamos o caso de um paciente com início insidioso e lentamente progressivo de declínio cognitivo, sintomas comportamentais, crises convulsivas do lobo temporal e pensamentos delirantes típicos dos delírios monotemáticos de identificação. A apresentação clínica associada aos exames complementares levaram ao diagnóstico de encefalite límbica relacionada a linfoma difuso de grandes células B. O paciente foi submetido a imunoterapia com altas doses de corticoide sem melhora clínica. Não foi realizado tratamento específico para o linfoma em função do paciente ter evoluído para óbito por choque séptico secundário a infecção respiratória nosocomial. Os delírios monotemáticos de identificação representam uma forma única e incomum de comprometimento cognitivo nas quais o paciente consistentemente identifica de forma errônea pessoas, lugares, objetos ou eventos. A síndrome de Capgras é o subtipo mais comum dessas desordens, sendo definida como a crença recorrente e transitória que um impostor substituiu uma pessoa familiar. Essas entidades são geralmente relacionadas com doenças neurodegenerativas e distúrbios psiquiátricos. Raros relatos da associação entre os delírios monotemáticos de identificação e doenças autoimunes como esclerose múltipla foram publicados, porém não há artigos abordando a correlação com encefalite límbica ou linfoma.

Los delirios en la anorexia nerviosa: una hipótesis neurobiológica (Segunda Parte) / The deliriums of nervous anorexia: a neurobiological hypothesis (second part)

La anorexia nerviosa (AN) es una enfermedad muy grave. Tiene un curso crónico y una morbi-mortalidad entre las más altas de las patologías psiquiátricas. Se caracteriza por una firme negativa a aumentar o recuperar peso, un miedo intenso a engordar y distorsiones de la imagen corporal. Estas ideas extrañas, exageradas en muchas ocasiones sorprenden por la firmeza con que son defendidas por las pacientes. "En algunos casos la desnutrición autoprovodada está asociada con creencias sobre la alimentación y la forma del cuerpo que son tan extremas que los psiquiatras las describen como delirantes o cuasi delirantes". De hecho, las características de la enfermedad la hacen comparable con algunos tipos de psicosis. De acuerdo con Bruch existe como un rasgo propio de la enfermedad la "negación delirante de la delgadez". A lo largo del trabajo se revisa una posible fisiopatología de estas ideas delirantes. Y se las compara con las ideas delirantes presentes en otras patologías. Para ello en la primera parte del trabajo se ha decripto la AN, su clínica y su fisopatología. En esta segunda parte se detalla la fisiopatología del síntoma delirio. Se concluye con una descripción de los delirios del tipo que se presentan en la anorexia nerviosa.

Nervous Anorexia (NA) is a very serious disease. It is a chronic condition and its morbidity is among the highest of psychiatric pathologies. It is characterized by a strong unwillingness to put on or regain weight, a deep fear to grow fat, and by distortions of the body image. These strange ideas, which in many circumstance are exaggerated, are striking in the sense that they are strongly held by the patients. At times, self-induced malnutrition is associated with beliefs about diet and silhouette, which are so extreme that Psychiatrists describe them as delirious or quasi-delirious. In fact, the characteristics of the disease make it possible to compare it with some types of psychosis. According to Bruch, there is a feature which is characteristic of the disease, namely, "the delirious denial of thinness". In this article, the author does a review of a possible physiopathology of these delirious ideas and compares them with the delirious ideas existing in other pathologies. In this second part the auhtor specifies the physiopathology of the delirium symptom and concludes with a description of the types of deliriums that manifest in nervous anorexia.

Capgras syndrome presenting in an adolescent girl in the Caribbean / El síndrome de capgras en una adolescente del Caribe

The case of a 16-year old Jamaican girl who presented to the psychiatric service of a general hospital with features of Capgras syndrome is presented. Her history, treatment, progress and relevant psychodynamic and neurocognitive issues are explored. This is the first known published case of an adolescent with Capgras syndrome from the Caribbean. The case highlights that the syndrome may occur in different cultural contexts and that clinicians should be sensitive to its existence in order to avert under-diagnosis or misdiagnosis.

Se presenta el caso de una joven jamaicana de 16 años que acudió al servicio de psiquiatría de un hospital general con los rasgos del síndrome de Capgras. Se explora su historia, tratamiento, progreso, problemas neurocognitivos y psicodinámicos relevantes. Éste es el primer caso publicado de que se tenga noticias, de una adolescente con el síndrome de Capgras en la región del Caribe. El caso pone de relieve que el síndrome puede ocurrir en diferentes contextos culturales, y que los médicos deben de estar sensibilizados con su existencia a fin de evitar un subdiagnóstico o un diagnóstico erróneo.

The Characteristics of Capgras Syndrome in Patients Diagnosed as Probable Alzheimer Disease

OBJECTIVE: We assessed the characteristics of Capgras syndrome in patients diagnosed as probable Alzheimer disease, and compared the clinical and imaging findings with other previous reports. METHODS: We obtained medical records of patients with Capgras syndrome diagnosed as probable Alzheimer disease in three hospitals from March 2009 to March 2010. The basic characteristics, neuropsychologic tests, brain imaging data were investigated. RESULTS: The mean age of the patients was 74.7 years old and all of the patients experienced this syndrome in relation to a spouse. Brain magnetic resonance imaging showed mild-to-moderate global atrophy, variable hippocampal atrophy in this study. The Mini-Mental State Examination mean scores were 18.78 and clinical dementia rating scores were from 1 to 2. All of the patients took medicine of acetycholine esterase inhibitors and two patients recovered with antipsychotic medication. Five patients had showed continuous Capgras syndrome, at the last follow-up day, for a year. CONCLUSION: Capgras syndrome patients in this study who were diagnosed as probable Alzheimer disease showed similar results to the individuals from other reports. More study is needed to estimate the numbers and characteristics of patients with this syndrome.

Clinical Features of Parricide in Patients with Schizophrenia / 신경정신의학

OBJECTIVES: This study aimed to describe the demographic, clinical and offense characteristics of the patients with schizophrenia who committed parricide. This study also investigated the risk factors of parricide by comparing with characteristics of stranger killers to prevent the tragic offense. METHODS: The sample consisted of 88 schizophrenic patients who committed homicide, 59 of them committed parricide and 29 of them killed strangers. Medical chart review regarding demographic and clinical characteristics as well as offense characteristics was conducted. The information that was difficult to be obtained through chart review was acquired by direct interview. RESULTS: Parricide group had earlier age of onset, more conflicts with the victims, more violence history toward victims and more offense provoking events than stranger killing group. Parricide group showed higher rate of past treatment than that of stranger killing group. However drug compliance at the time of offense was low in both groups. Offense provoking events in the parricide group included blaming, threatening a forced hospitalization or medication toward patients. The most common psychiatric symptoms associated with homicide were threat/control override (TCO) symptoms, delusional perception, commanding auditory hallucination and especially with parricide, Capgras syndrome (CS). CONCLUSION: Interaction between psychotic symptoms such as TCO, CS and underlying emotion toward victims are likely to be associated with parricide. Mental health professionals are expected to play an important role in preventing this tragic offense by active history taking of the violence and effective management with active caregiver education/intervention of intrafamilial conflicts.

Clinical features of Alzheimer\u2019s patients with Capgras syndrome

Background: Capgras syndrome is a special psychotic feature of Alzheimer\u2019s Disease (AD). It is the most common disorder among 4 primary delusional misidentification syndromes in AD. However, there\u2019s not been that much research done into this syndrome in Vietnam. Objectives: To describe the clinical appearances of Capgras syndrome and its effect on the severity and progress of AD. Subjects: 35 patients with diagnoses of AD according to DSM-4 criteria, treated at the National Institute of Mental Health, National Psychiatric Hospital and Hanoi Psychiatric Hospital over a period of 5 years (1997-2002). They were divided into 2 groups with and without Capgras syndrome. Method: Descriptive, prospective and longitudinal study. This was small size clinical study, so p\u2013value was used to compare 2 groups of patients. Results: Of the 35 AD patients, 8 patients (22.8%) had Capgras syndrome. Capgras syndrome was seen more frequently in women (6/8 patients) at the late episode of the disease. Its features were similar to descriptions by journals on the subject, 5 out of 8 patients had delusions. Disability on daily life and psychotic, conductive, affective disorders were seen more frequent and severe on patients with Capgras syndrome. Paraclinical findings showed that in patients with Capgras syndrome, the lesions were more predominant in the right hemisphere. Conclusion: Capgras syndrome was usually accompanied with behavioral, affective disorders. Especially, it increases cognitive impairment of patients. Further studies on the relationships between Capgras syndrome and special lesions in the brain are required.

Síndrome de Capgras en enfermedad de Alzheimer: presentación de 2 casos / Capgras syndrome in Alzheimer's disease: two cases

Se presentan dos casos de síndrome de Capgras (SC) en mujeres con enfermedad de Alzheimer. Ambas tenían la convicción delirante de que su esposo no era tal sino que un impostor; la primera en forma consistente y duradera, la segunda en forma fluctuante. La revisión de la literatura permite comparar el SC con la paramnesia reduplicativa y el “jamais vu” y contrastarlo con la prosopagnosia. La fisiopatología del SC dependería de que al percibir un rostro se procesan en forma paralela a) los detalles visuales que permiten reconocerlo como un rostro característico (capacidad que se pierde en la prosopagnosia), y b) la respuesta emocional que se pierde en el SC. La pérdida de familiaridad de una persona conocida induce la sensación de que se trata de un impostor. Los estudios de imágenes funcionales permiten suponer que en el procesamiento de los rostros y voces familiares participa en forma destacada la parte posterior del girus cingulado.

Two cases Capgras syndrome (CS) are presented in women with Alzheimer’s disease. Both patients had the conviction that their husbands had been supplanted by an impostor; in the first one consistently and permanently, and the second one in a fluctuating form. Revision of the literature allows to compare CS with reduplicative paramnesia and “jamais vu”; and contrast these with prosopagnosia. The physiopathology of CS can be explained by assuming that during face recognition two processes are participating in parallel: a) the recognition of visual details, that allow identification of a face (which is lost in prosopagnosia), and b) the emotional response, which is lost in CS. The loss of familiarity of a known person induces the sensation of an impostor. Functional imaging studies suggest that the posterior cingulate gyrus has a predominant role in the processing of familiar faces and voices.

Síndromes delirantes de falso reconhecimento: curiosidade ou chave para um novo conhecimento psiquiátrico? / Misidentification syndromes: curiosity or a key for a new psychiatric knowledge?

Objetivos: Partindo das definiçoes das Síndromes Delirantes de Falso Reconhecimento, tendo como principais modelos as Síndromes de Capgras e Frégoli, os autores fizeram uma revisao dos aspectos psicopatológicos envolvidos nesses quadros. Métodos: Foi realizado um levantamento da Literatura fenomenológica clássica e posterior adaptaçao de um modelo neuropsicológico que aprofunde o entendimento desses quadros, geralmente negligenciados pela literatura ou tratados como extravagências de psicopatólogos antigos. Conclusoes: O entendimento das Síndromes Delirantes de Falso Reconhecimento, no entender dos autores, pode ser de grande valia para formar novos conceitos no estudo dos quadros psiquiátricos delirante-alucinatórios maiores, principalmente das correlaçoes entre Sensaçao, Percepçao e Atribuiçao de Significado.

Síndromes neuropsiquiátricos por lesión en el hemisferio cerebral derecho / Neuropsychiatric syndroms produced by lesions in the right brain hemisphere

El estudio de la dominancia cerebral desempeña un papel importante en el contexto de las desórdenes de las funciones cerebrales superiores. No sólo las neuropsicológicas se encuentran lateralizadas sino también las neuropsiquiátricas. El objetivo de nuestro trabajo es la presentación de tres pacientes con trastornos psiquiátricos secundarios a una lesión del hemisferio cerebral derecho. El caso 1 es una paciente con un síndrome de Capgras, los casos 2 y 3 un síndrome de hemidespersonalización. Es frecuente que lesiones en el hemisferio derecho sean responsables de síndromes neuropsiquiátricas. El hecho de que ni las alteraciones funcionales, ni las estructurales puedan explicar por sí solas todas las características de los síndromes en estos pacientes, hace suponer que es necesaria la conjunción de ambas em proporciones variables según los casos. Probablemente aparezca en un sujeto con terreno paranoide con ligeros desórdenes perceptivos; o a la inversa, en un paciente con importantes alteraciones perceptivas como nuestros pacientes (visual en el caso 1; y sensitivas en los casos 2 y 3), bastará un pequeño trastorno afectivo para que surja la idea delirante.

Síndrome de Capgras por lesión cerebral posterior derecha / Capgras Syndrome for cerebral lesion right hemisphere stroke

En 1923 el psiquiatra francés Joseph Capgras describió un cuadro clínico cuyo síntoma central era la firme creencia del paciente de que personas muy conocidas, generalmente familiares, habían sido reemplazadsa por dobles, impostores. Los pacientes reconocían su exacta similitud fisonómica, si bien negaban su identidad. Se describe una mujer de 59 años, diestra, sin historia psiquiátrica o neurológica previa, que luego de un accidente cerebrovascular isquémico cerebral posterior derecho (RM cerebro) refiere compartir algunos momentos de su actividad diaria con su marido, y más tarde con otra persona de igual fisonomía (que en realidad era su marido) a quien se refería como "el otro Manolo" o el "muchacho". En la evaluación cognitiva mostró una hemi-inatención izquierda, trastornos visuoperceptivos y visuoconstructivos, alteraciones mnésicas y severos fenómenos perseverativos. Desde su descripción original esta patología fué considerada como una manifestación exclusivamente psiquiátrica. A principios de la década del 70 comenzaron a publicarse trabajos que la asociaban a daño cerebral orgánico, referido casi siempre a lesiones hemisféricas derechas. Se relacionó a los desórdenes en la representación afectiva o de familiaridad o a fallas en el procesamiento perceptivo preconciente, ligados a una base paranoide como disparadores de este síndrome

El síndrome de Capgras: estudio clínico de nueve casos / The Capgras syndrome: a clinical study of 9 cases

The Capgras' syndrome is the delusional belief that a person or object has been replaced by an identical duplicate, usually with evil intent. A clinical, psychometric and electroencephalographic study of 9 patients afflicted with this syndrome and its variants was performed. The clinical course and the therapeutic response were assessed during the follow up that run between 2 and 11 years. The aim of this paper is to discuss the etiologically relevant psychodynamic, organic and mythological issues and to postulate an explanatory model that integrates limbic mechanisms, affect recognition processes, psychodynamic factors and anthropological aspects

Aspectos psiquiátricos e neurológicos da síndrome de Capgras e de outros distúrbios de identificaçäo correlatos / Psychiatric and neurologic aspects of Capgras syndrome and other disorders of correlates identification

Foram avaliados 260 relatos de caso sobre distúrbios de identificaçäo. Destes, 174 pacientes apresentavam síndrome de Capgras, 18 síndrome de Frégoli, 11 intermetamorfose, 17 síndrome do duplo subjetivo, 17 paramnésia reduplicativa e o restante combinaçöes de dois ou mais destes. O sexo e a idade média dos pacientes näo mostraram diferenças significativa. Sintomas psicóticos foram significativamente mais prevalentes (p < 0,005) em pacientes com sídrome do duplo subjetivo. Esquizofrenia, distúrbio de humor e síndromes mentais orgânicas foram menos freqüentemente descritas em pacientes com paramnésia reduplicativa. Estes, por sua vez, apresentavam maior prevalência de traumatismo craniano (p < 0,05) e lesöes focais de hemisférico cerebral direito (p < 0,005). A diferença entre o quociente de inteligência verbal e de desempenho encontrada sugere que os pacientes com paramnésia reduplicativa (p < 0,01) apresentam disfunçäo de hemisférico direito mais severa que os demais grupos. A identificaçäo errônea de outras pessoas (síndrome de Capgras, Frégoli e intermetamorfose) ou de si mesmo (síndrome do duplo subjetivo) pode ser expressäo de um quadro psicótico de natureza psiquiátrica ou orgânica, enquanto paramnésia reduplicativa está mais comumente associada a doenças neurológicas, particularmente quando estas afetam o hemisfério direito. A prevalência dos distúrbios de identificaçäo é possivelmente subestimada

Síndrome do duplo subjetivo e síndrome de Capgras: relato de caso e revisäo da literatura / Syndrome of subjective doubles and Capgras syndrome: case report and literature review

Os autores apresentam o caso de paciente com psicose paranóide crônica que desenvolveu o delírio de um duplo de si mesmo, constituindo a "sindrome do duplo subjetivo". Ao mesmo tempo, apresentava delírios de substituiçäo envolvendo uma pessoa de sua família e um político do cenário internacional, configurando a "síndrome de Capgras". O caso é descrito e comentado à luz dos dados de revisäo de literatura, com ênfase nos aspectos do diagnóstico diferencial, investigaçäo de fatores orgânicos, testagem neuropsicológica e aspectos psicodinâmicos supostamente relacionados à especificidade do delírio, objetivando uma abordagem integrada destes quadros

El síndrome de Capgras: revisión general a propósito de tres casos clínicos / Capgras syndrome: general review apropos of three cases

A propósito de tres casos, se revisa el Síndrome de Capgras de acuerdo con la literatura actual sobre el mismo y las características clínicas de los casos presentados, se hace una breve discusión sobre las peculiaridades clínicas del cuadro así como algunos aspectos psicopatológicos y etiopatologías de este síndrome delusional de suplantamiento de identidad.

Upon three clinical cases, CapgrasÆ Syndrome is reviewed according to the up-to-date literature on the subject and the clinical characteristics of the case-histories presented. A brief discussion is made as to clinical traits as well as to some psychopathologic and ethiopathogenic aspects of this delusional misidentification syndrome.